Papulosquamous diseases: A review

IOURNAL o f t i l e

AmeRicaN ACaDemY OF

DerMaTOLOGY VOLUME 12 II

II I

Continuing

NUMBER 4

APRIL, 1985

I Illl I1

medical

education

Papulosquamous diseases: A review* Barbara J. Fox, Captain, MC, USA,** and Richard B. Odom, M.D.*** San Francisco, CA Papulosquamous diseases are a heterogeneous group of disorders whose etiology primarily is unknown. The nosology of these disorders is based on a descriptive morphology of clinical lesions characterized by scaly papules and plaques. The major entities in this group include psoriasis, parapsoriasis (including pityriasis lichenoides et varioliformis acuta), lichen planus, llchen nitidus, lichen striatus, pityriasis rosea, pityriasis rubra pilaris, seborrheic dermatitis, and the Gianotti-Crosti syndrome. Many other conditions may become papulosquamous and should be considered in the differential diagnosis. (J AM ACAD DERMATOL12:597-624, 1985.)

Papulosquarnous diseases include many common dermatologic conditions and a few less common ones. The major entities in this group to be reviewed are listed in Table I. Psoriasis ! and parapsoriasis 2 have been reviewed in previous CME articles and have been excluded. The com-

mon denominator of all of these dermatoses is that they are characterized by scaly papules and plaques. Many other conditions also may become papulosquamous, and adverse reactions to many drugs may produce papulosquamous eruptions. It is important to consider all possible dermatoses in the differential diagnosis of a papulosquamous eruption.

"oAAD~ The CME articles are made possible through an "-':'~.... educational grant from Syntex Laboratories, Inc. From the Dermatology Service, Department of Medicine, Letterman Army Medical Center, Presidio of San Francisco. Reprint requests to: Technical Publications Editor, Letterman Army Medical Center, Presidio of San Francisco, CA 94129. *The opinions or assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the Department of the Army or the Department of Defense. **Present duty station is William Beaumont Army Medical Center, El Paso, TX 79920. ***Present address: Department of Dermatology, University of California School of Medicine, San Francisco, CA 94143.

LICHEN PLANUS Lichen planus (LP) is an inflammatory dermatitis producing a characteristic eruption that is primarily papular in origin. The distinctive lesion is a polygonal, violaceous papule. Its slightly scaly surface consists of fine white lines catled "Wickham's striae," or small gray-white puncta (Fig. 1). L P usually is highly pruritic; however, secondary excoriations are rare. 3"5 Both sexes are affected approximately equally. Disease occurrence is most common between the ages of 30 and 60

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Abbreviations used EPS erythrokeratodermia progressiva symmetrica GLPLS Graham Little-Piccardi-Lassueur syndrome HBsAG hepatitis B surface antigen HBc hepatitis B core (antibody) HBeAG hepatitis Be antigen LN lichen nitidus LP lichen planus LPP lichen planopilaris LS lichen striatus PAC papular acrodermatitis of childhood PAS papulovesicular acrolocated syndrome PR pityriasis rosea PRP pityriasis rubra pilaris RBP retinol-binding protein

years; children and the elderly rarely are aff e c t e d Y ,6 While its incidence varies in different geographic locales, LP has been reported to represent from 0.14% to 1.2% of all dermatologic conditions seen by dermatologists? '7'8 Wilson 9 in 1869 was the first to describe LP, also known as "lichen ruber planus," in some European countries. Although during the last century a great deal of work was accomplished in recognizing and categorizing its numerous variants, little is known concerning the etiology of LP. Several theories have been proposed to explain the causation of LP but have in fact little support. The suggestion that LP is due to either a viraP ° or a bacterial 1~ infectious agent is based mainly on identification of a morphologic structure on microscopy. Presence of a consistent immunofluorescent pattern suggests an immunologic etiology. 12,~3 Direct immunofluorescence shows numerous ovoid globular deposits (representing the "colloid bodies") of IgM, IgG, IgA, and/or complement just below the dermoepidermal junction. There may also be a linear deposit of fibrinogen at the dermoepidermal junction. Indirect immunofluorescence is negative. A LP-like eruption may occur following bone marrow transplantation as a chronic form of graft-versus-host reaction. 14.15 The skin and mucous membrane lesions, as well as the histology may be similar to LP, and the basal cell damage is thought to be the result of an

Journal of the American Academy of Dermatology

immunologic reaction that has not yet been fully elucidated. 16 Numerous accounts show initial onset or relapse of LP following periods of emotional stress, 3'5'6't7 but a causative relationship based on this subjective factor is difficult to establish. Another theory is that certain individuals are genetically predisposed to acquiring LP. 5,6 Although uncommon, familial cases of LP do occur; it has been proposed that these patients may have a c o m m o n human leukocyte antigen (HLA) type. C o p e m a n and coworkers TM found an increased incidence of HLAB7 in ten cases of familial LP. Veien et al, '9 in a study of eighty-nine nonrelated patients, found no significant association between any particular HLA type and LP; however, they did see a slightly greater incidence of HLA-A3 and HLA-B5. Mahood 2° stated that the number of familial cases studied by tissue typing is too small to draw any conclusions concerning an association with specific HLA types. Several neurologic conditions have been seen in some cases of LP, 3 but this association does not appear to be a c o m m o n one. Finally, several drugs have been implicated in causing a LP-like eruption, including quinacrine,~ streptomycin, para-aminosalicylic acid, methyldopa, quinidine, phenothiazine, thiazides, chloroquine, 6 chlorpropamide, heavy metals (gold, bismuth), 4 color-photographic developer (paraphenylenediamine salts), 22 penicillamine, 23,24 and captopril.55 Lichen planus may develop rapidly over several days into an acute generalized dermatitis. The flexor surfaces of the wrists and forearms are frequently involved first; other c o m m o n areas of involvement are the lumbar area, ankles, glans penis, anterior aspect of the lower legs, and the dorsal surfaces of the hands. S o m e papules are umbilicated. The papules may be scattered or may become grouped into larger lesions. Koebner's phenomenon is common. Approximately 15% to 25% of patients who present with oral L P never have skin lesions. 3 Mucous membranes are affected in more than half of the patients. 4'6 The buccal mucosa and the tongue are most often affected but the lips, gums, palate, conjunctivae, larynx, anus, genitalia, gastrointestinal mucosa, bladder, and tympanic membrane have also been involved. The mucosal lesions consist o f lacy, re-

Volume 12 Number4 April, 1985 ticulated white streaks, white papules and plaques, and atrophic and ulcerated patches (Fig. 2). Chronic erosive or atrophic oral LP may predispose to an oral squamous cell carcinoma, but the risk is controversial and probably very low. 26 An interesting triad of oral LP, diabetes mellitus, and hypertension is referred to as Grinspan's syndrome. 27 Screening patients with oral LP for hypertension and abnormal glucose levels may be worthwhile. Approximately one fourth of male patients with LP have involvement of the genitalia3 (Fig. 3). Lesions are most commonly seen on the glans penis and may present as typical papules or in an annular configuration. Occasionally, the glans penis has erosive lesions and is the only site of involvement. 28 Nails are affected in about 10% of cases. 3'~'6'29 Symptoms include thinning of the nail plate, longitudinal ridging and splitting, subungual hyperkeratosis, onycholysis, red or brown discoloration, pterygium formation, or complete loss of the nail plate (Fig. 4). Pterygium formation results from fusion of the proximal nail fold with the nail bed leading to a focal loss of the proximal nail plate. Pterygium formation is fairly distinctive for LP but may also result from trauma, infection, or peripheral vascular disease. Complete loss of the nail of the great toe is most common. Scher et al3° believe that twenty-nail dystrophy frequently represents a variety of LP, LP may affect the palms and soles, producing hyperkeratotic, yellowish papules, ~ which usually lack the characteristic morphology of typical LP papules and often are nonpruritic. Diagnosis of LP based on these palmoplantar lesions can be difficult, unless other areas exhibit more characteristic findings. Acute LP can usually be expected to resolve in 6 to 18 months. 6 Healed lesions often leave a hyperpigmentation that may take months to clear. Chronic LP, which can develop insidiously or from acute forms, may persist for more than a decade. Oral LP, hypertrophic LP, and lichen planopilaris particularly tend to be chronic. Several distinctive variants of LP need to be recognized, since lack of characteristic changes in these cases makes diagnosis difficult. 3'5'6J7 These variants include:

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Annular LP. Annular lesions usually form from a ring of typical LP papules that spread peripherally and produce central clearing (Fig, 5). Annular lesions are common on the glans penis but may occur anywhere. Linear LP. This uncommon variant consists of a linear group of typical LP papules, most often seen in children. Occasionally it may be zosteriform. It is important to consider linear LP in the differential diagnosis of other linear dermatoses. Hypertrophic L P (lichen planus verrucosus). Occurring mainly on the shins, ankles, and soles, this variant consists of highly pruritic, lichenified, scaly, violaceous, or hyperpigmented plaques (Fig. 6). The lesions are usually symmetric, tend to be chronic, and may be the only manifestation of LP. Malignant degeneration has occurred. Atrophic LP. Atrophic lesions may occur on mucous membranes. In unusual cases, atrophy of LP papules may produce atrophic white spots. This variant needs to be distinguished from lichen sclerosus et atrophicus and guttate morphea. Vesieulobullous LP. Vesicular or bullous lesions may develop on preexisting LP lesions or on previously uninvolved skin. The term "lichen planus pemphigoides" has been used to describe bullous LP. However, Mora and co-workers31theorize that LP pemphigoides is not a variant of bullous LP. Bullous pemphigoid developed in their patients soon after they had acquired LP. Direct and indirect immunofluorescent studies confirmed coexistence of the two diseases. The authors 31 suggest a possible causal relationship in these instances, perhaps related to an immunologic phenomenon. LP aetinieus (LP tropicus, LP pigmentosus). This variant occurs mainly in the tropics (especially the Middle and Far East), on sun-exposed surfaces of children and young adults. Lesions may be pigmented, dyschromic, or granuloma annulare-like, and usually are only mildly pruritic. LP actinicus is histologically distinguishable from discoid lupus erythematosus. LP erythematosus. Nonpruritic, soft, red papules may be observed in older patients, especially on the forearms. Additional typical LP lesions may or may not be present. Although histologically the lesions show typical features of LP, whether or not it represents a true variant of LP is questionable.

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Figs. 1, 3, 5.7. For legends, see opposite page.

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Fig. 2. LP of the buccal mucosa.

Lichen planopilaris (LPP, follicular LP). LP of the hair follicles presents as acuminate, keratotic, follicular papules, primarily on the scalp, although papules may appear on any hair-bearing surface (Fig. 7). Affected areas may also exhibit scaling, atrophy, and alopecia. Scarring and permanen t alopecia sometimes result, and LP is one of the causes of pseudopelade of Brocq22 Lichen planopilaris (LPP) may occur alone or with more characteristic LP lesions elsewhere. A variant of LPP is the Graham Little-Piccardi-Lassueur syndrome (GLPLS). This syndrome was recently reviewed by Horn et al, 33 who also reported on the immunofluorescent findings in LPP and GLPLS. It consists of cicatricial scalp alopecia, follicular keratotic lesions of the glabrous skin, and, at times, noncicatricial alopecia of the axilla and groin. The direct immunofluorescence of both LPP and GLPLS is similar to that of LP except that the

Fig. 4. LP of the nails demonstrating atrophy and irregular longitudinal ridging and grooving of the nail plates.

Fig. 1. LP. Note the violaceous color, the polygonal-shaped papule, and the linear lesion demonstrating Koebner's phenomenon. Fig. 3. LP on the glans penis, an area commonly affected. Fig. 5. Annular LP. Fig. 6. Hypertrophic LP on the lower extremities of a 6-year-old girl. Wickham's striae can be easily identified in the large lesion near the knee. Fig. 7. LPP on the lower extremity. (Courtesy Dr. Robert F. Findlay, Augsburg, Germany.)

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Fig. 8. LN above the umbilicus. (Courtesy Dr. William D. James, Washington, DC.) changes occur in the superficial follicular epithelium. Ulcerative LP. An ulcerative form of LP is recognized that consists of chronic bullae and ulcerations of the soles of the feet, and sometimes of the mouth and other mucosae, associated with cicatricial alopecia o f the scalp and loss of toenails, a''Tm More typical LP lesions on other parts of the body may be present or absent. The lesions on the feet can be painful and disabling and have a definite risk of development of a squamous cell carcinoma in the chronic ulcerations. 17,35Excision and split-thickness skin grafting of the affected areas offer the best form of therapy. 34.35 The histopathology of LP is fairly diagnostic. It consists of hyperkeratosis (with the absence of parakeratosis); focal hypergranulosis ("beading"); irregular acanthosis (producing a "sawtoothed" appearance); liquefaction degeneration of the basal cell layer; and a bandlike upper dermal lymphohistiocytic infiltrate.36 There may be a considerable incontinence of pigment due to basal layer damage. Colloid bodies (hyaline, cytoid, or Civatte bodies) are commonly present in the deep epidermis and the superficial dermis. Small separations between the epidermis and the dermis may be present and are called "Max Joseph spaces." These separations m a y lead to subepidermal vesicle formation.

Hypertrophic LP lesions show acanthosis, papillomatosis, and hyperkeratosis. Oral lesions often show parakeratosis rather than hyperkeratosis and frequently have an atrophic epidermis. Lichen planopilaris lesions show a similar lichenoid infiltrate around the hair follicles. In early lesions of LPP, the follicle is dilated and filled with a keratotic plug; later, however, there is destruction of the follicle. Evaluating the efficacy of different forms of treatment in LP is difficult, since LP tends to regress spontaneously after varying amounts of time. In mild cases, treatment should be symptomatic: antihistamines for pruritus and topical corticosteroids for their antipruritic and anti-inflammatory effects. Severe, acute cases may benefit from a tapered course of systemic corticosteroids for 2 to 6 weeks. Relapses may occur, however, and chronic systemic steroid use should be avoided. One study 37 suggests that oral griseofulvin may be beneficial. Topical vitamin A acid m a y also be useful in treating some cases of cutaneous LP? ~ Oral LP lesions are best treated with a potent corticosteroid mixed with an oral protective base, Orabase, or intralesional corticosteroids. Hypertrophic LP lesions may also benefit f r o m intralesional steroids, or corticosteroids under occlusion or tar preparations. Patients under a great deal of stress frequently show improvement when their

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Fig. 9. LS of the buttock and posterior aspect of the thigh in a child.

Fig. 10. LS of the abdomen and upper aspect of the thigh in an infant.

Fig. 11. Lesions of PR showing the "collarette" scale that is attached at the periphery and free in the center of the lesion.

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r

Fig. 12. "Inverse,' PR involving the intertriginous area of the groin. Fig. 13. Typical acuminate follicular papules of PRP. Fig. 14. Yellow-orange hyperkeratosis of the palms in PRP. Fig. 15. Severe seborrheic dermatitis in an infant involving the 'scalp and upper torso. Fig. 17. Lichen aureus on the lower extremity. (Courtesy Dr. Robert F. Findlay, Augsburg, Germany.)

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emotional environment is altered. Ortonne et al ~ reported that oral photochemotherapy (PUVA) was effective in one of their patients with LP. A study by Mahrle et al 4° demonstrated that oral etretinate therapy may have beneficial effects in some patients with LP, but the response is less dramatic than etretinate therapy used in some other dermatoses. LICHEN NITIDUS Lichen nitidus (LN) is an uncommon chronic eruption of numerous tiny, discrete, usually fleshcolored, shiny papules most commonly located on the penis, arms, forearms, and abdomen. It was described by Pinkus, first in 1901, and again in 1907. 41 The distribution of LN is most often localized, but in some cases it may become generalized. 42,43Its occurrence has been infrequently reported on the palms and soles 44'45 and on mucous membranes. 46 Some authors 47 believe that there is no age predilection, whereas others 48,49report it as being more prevalent among children or young adults. A retrospective histopathologic study by Lapins et a142 demonstrates an age range of 5 to 58 years. Although LN usually is asymptomatic, it sometimes is mildly pruritic. 42 Reliable epidemiologic data are difficult to accumulate because of the relative rarity of LN, but all races and both sexes appear to be susceptible. Although LN has been reported to occur with lichen planus, 5° it has not been associated with any systemic diseases. 47 KAnt et al, ~1 however, described a patient who had Crohn's disease in whom LN later developed. Since both LN and Crohn's disease have granulomatous infiltrates, they suggested that LN may be a cutaneous manifestation of regional ileitis. Individual papules are uniformly pinhead size and usually are flat, with a shiny surface (Fig. 8). Some are dome-shaped, 49 and many have a central depression. 47 Although most papules are skin-colored, others are pink, yellowish, red-blue, or hyperpigmented. 42 In blacks, the eruption is often more easily recognized because the papules tend to be hypopigmented? 2 Jetton et a153 reported a patient who had vesicular and hemorrhagic lesions intermixed with typical LN papules. Although grouped papules tend to remain as distinct papules, coalescence of papules in the generalized or confluent type may form plaques. 47 On elbows and

Fig. 16. Lichenoid papules on the lower extremities o f a patient with Gianotti-Crosti syndrome.

knees these plaques may appear to be psoriasiform. Lichen nitidus is one of the many diseases showing the Koebner phenomenon?2,54 This isomorphic response rarely is seen in patients with LN, perhaps because it is usually nonpruritic, and patients are, therefore, less likely to traumatize their skin by scratching. Arndt 47 commented that in patients with LN, the nails may become pitted, ridged, thick, and brittle. We could find no bther reference to nail changes so nail involvement apparently is unusual. Frequently the diagnosis of LN is made clinically without the aid of a biopsy. If, however, the diagnosis is questionable or confirmation is needed, the histologic picture is very distinctive, s5 In their review of 43 cases of LN, Lapins et a142 outlined the main histologic features. Hematoxylin and eosin staining usually shows a small dermal papule with overlying epidermal atrophy and frequently a parakeratotic " c a p " over the center o f

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the lesion. The granular layer is thin or absent. Liquefaction degeneration of the basal layer is uniformly present and may be accompanied by pigment incontinence. The hydropic degeneration may produce a focal separation between the epidermis and the dermis (a Max Joseph-like space). The bulk of the papule is formed by a focal accumulation of inflammatory cells occupying a widened dermal papilla. The infiltrate consists of lymphocytes and epithelioid cells and occasional multinucleated giant cells of the Langhans type. This granulomatous infiltrate is closely apposed to the epidermis. The epidermis has elongated, "clawlike" rete ridges that appear to grasp the dermal infiltrate as in a "baseball and glove." Edema is present within the infiltrate, and small blood vessels show endothelial proliferation. Bardach 56 described a recent case of perforating LN. Although perforating LN has not been observed previously, its occurrence is not too surprising because of epidermal thinning and close proximity of dermal infiltrate to the epidermis. Another new finding in LN has been reported by Clausen et al, 57 who used electron microscopy to study a skin biopsy specimen from a patient with LN and found a significant number of Stzary cells in the dermal infiltrate. This case probably represents another example of a dermatosis whose infiltrate contains reactive lymphocytes with cerebriform nuclei. The papules of LN often are so distinctive that a clinical diagnosis is not difficult. However, this is not always the case, and various other entities could be considered in the differential diagnosis. These entities include verruca plana, LP, keratosis pilaris, lichen scrofulosorum, lichen spinulosus, lichen amyloidosus, lichen myxedematosus, Darier's disease, sarcoidosis, lichenoid secondary syphilis, folliculitis, id eruption, and lichen simplex chronicus. A biopsy should allow exclusion of these disorders and lead to a proper diagnosis. The cause of LN currently remains a mystery. At first it was believed that LN might represent a tubercular lesion or tuberculid. 47 Many studies investigated this possibility, but no connection could be found. Over the years, many authors have debated at length on whether or not LN is a variant of LP. Both entities have often been found to coexist in the same patient; one article 5° claims that

lesions identical to LN can be found in 25% to 30% of patients with LP. The two diseases show several similarities: 47 (1) LP may begin as small papules similar to LN, but these papules usually progress to larger lesions; (2) both may show a Koebner phenomenon; (3) both may clear spontaneously, although LP usually resolves in a shorter time; (4) both show a lichenoid lymphohistiocytic infiltrate; and (5) both have liquefaction degeneration of the basal cell layer. There are also many significant differences: (1) LP is usually intensely pruritic, whereas LN is only occasionally mildly pruritic; (2) LN lacks Wickham's striae and is usually not violaceous in color; (3) mucous membrane involvement is common in LP, but uncommon in LN; (4) LN has a parakeratotic cap, whereas LP rarely shows parakeratosis; (5) EP has hypergranulosis, whereas LN has a thin or absent granular layer; (6) LN has a granulomatous-type of infiltrate, sometimes with Langhans giant cells; (7) LN may show transepidermal elimination; and (8) direct immunofluorescence of LP shows numerous globular deposits of IgG, IgM, IgA, and/ or complement beneath the dermoepiderma! junetion, ss but no immunoglobulins have been found in LN. 57,59We believe that LN is a distinctly separate entity that has been recognized as such for over 80 years. Because of its infrequent occurrence, LN is a difficult disease to investigate. LN may clear spontaneously after one or several years, or it may persist indefinitely. 47 In children, no therapy is indicated because most cases eventually clear spontaneously. However, if significant pruritus is present, the papules should be treated with appropriate antihistamines and a topical corticosteroid. A1Tldt47 reported that potent corticosteroids under occlusion may induce flattening or disappearance of lesions, and that systemic corticosteroids may be beneficial in generalized forms of LN. L I C H E N STRIATUS

Lichen striatus (LS) is an uncommon, self-limited, unilateral, linear dermatosis of unknown etiology, ordinarily seen in children from 5 to 15 years of age, but it can occur at any age. 6°-62Girls are affected two to three times more frequently than boys. 6°,~ Staricco 6° in 1959 reviewed the history of LS.

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The first case was probably presented by Balzer and Mercier at the French Society of Dermatology in 1889 under the name of "lichenoid trophoneurosis." Other names used in the past include6°,63 zonal dermatosis, linear dermatosis, linear neurodermatitis, linear lichenoid dermatosis, neuroder. mite zoniforme, strichformige eruption, lichenoid eruption, systematized lichenification, and linear eczema. The term "lichen striatus" has been used in the European literature and was suggested by Senear and Caro 63 to be the most appropriate term in their review in 1941. LS usually erupts suddenly, develops fully over days to weeks, and then undergoes spontaneous involution after several months to a year or m o r e . 6°'63 It first appears as discrete pink, red, or flesh-colored, flat-topped, lichenoid papules, often with a fine silvery scale. These papules coalesce into a continuous or interrupted linear band 1 to 3 cm wide, in some cases extending the entire length of an extremity and onto the trunk (Figs. 9 and 10). The lesions most commonly occur on the extremities or neck but may appear on any part of the body. Multiple lesions 62,64 and bilateral involvement6s are rare. Papules may be hypopigmented in dark-skinned persons; after involution, a temporary postinflammatory hypopigmentation may occur. While LS is usually asymptomatic, it occasionally is pruritic. Eight cases of LS with associated nail changes have been reported. 63,66-69 When LS lesions extend right up to the proximal nail fold, longitudinal ridging, splitting, fraying, onycholysis, and total nail loss may be observed. The differential diagnosis of LS includes61.62,64,7° nevus unius lateris, linear LP, linear psoriasis, linear Darier's disease, linear isolated LN, 71 linear lichen simplex chronicus, inflammatory linear verrucous epidermal nevus, linear porokeratosis, tinea corporis, and verruca plana. The clinical course of LS is perhaps its most distinctive feature, but, if necessary, other entities in the differential diagnosis may be excluded by histologic examination. Pinkus 6°'7z in 1904 provided the first histopathologic description of LS. In 1975, Reed et al7~ provided an extensive review. The histology of LS is not specific and, in fact, may vary, depending upon the age of the lesion as well as within different areas of the same lesion. ~4,73Basically, LS

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shows a lichenoid reaction in the dermis with secondary epidermal changes.71.74.7~There is a chronic perivascular or bandlike infiltrate in the papillary dermis. The epidermis may exhibit intercellular and intracellular edema, exocytosis, parakeratosis, and focat separation of the basal ceils in areas where the dermal infiltrate abuts the epidermis. Dyskeratotic keratinocytes, similar to the corps ronds in Darier's disease, may be found in the granular and horny layers in up to one half of cases, 6°,74,75 Some areas of LS are histologically indistinguishable from LP, and when hair follicles are involved with the lichenoid reaction, differentiation from LPP becomes difficult. 7~Old lesions may resemble LN. The etiology of LS is unknown. As in other linear dermatoses, the factors involved in determining the linearity of the disease are also unknown. Various theories that have been proposed to explain this phenomenon have been reviewed in detail by Senear and Caro. 6~ Peripheral nerve or vessel distribution, or embryologic cleavage lines, cannot account for all cases. Whatever the mechanism, LS seems to develop in a "locus minoris resistentiae." Treatment of LS is usually unnecessary, unless pruritus is significant, or the location is a cosmetic concern. In these cases, topical corticosteroids under occlusion or intralesional steroids have been reported to hasten resolution. 61 PITYRIASIS ROSEA

Pityriasis rosea (PR) is a common, self-limited papulosquamous eruption primarily affecting children and young adults. Although PR usually occurs in persons 10 to 40 years o l d , 7678 it has been observed at almost any age. 79 B o t h sexes are affected approximately equally, and there is no racial predilection, The incidence of PR is roughly 1% of patients seen by dermatologists. 76,Ts,s°The peak incidence of PR has variably been reported as winter, spring, or autumn. 7~,77,8°The only clear conclusion that can be drawn from the literature is that the incidence is definitely lower during the summer months. PR was first described by Gibert in 1860. '1 Its clinical features are so characteristic that careful observation is the most reliable method for obtaining a diagnosis. The eruption generally begins

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Table I. Papulosquamous diseases-major entities Psoriasis Parapsoriasis 1. Large-plaque parapsoriasis 2. Small-plaque parapsoriasis 3. Pityriasis lichenoides a. Pityriasis lichenoides et varioliformis acuta b. Pityriasis lichenoides chronica Lichen planus Lichen nitidus Lichen striatus Pityriasis rosea Pityriasis rubra pilaris Seborrheic dermatitis Gianotti-Crosti syndrome

with a "herald or mother patch," which was first described by Brocq. 82 This patch, a round or oval erythematous lesion 1 to 5 cm in diameter, may be flat or slightly raised. The center may show cleating and the edge is often scaly. Although commonly found on the trunk, the herald patch can be found on any part of the body. Following an interval of a few days to a week or more, crops of papulosquamous lesions erupt in a generalized fashion (Fig. 1 1). These discrete lesions are similar to, but smaller than, the herald patch, and they are usually located on the trunk, neck, upper arms, and legs, tending to spare the sun-exposed areas. 77 The long axes of these oval lesions run parallel to the ribs, producing a "Christmas tree" pattern on the chest or back. The scaling of each lesion is typically a "collarette" scale that is free at the center and attached at the periphery of the lesion. 79 The eruption persists anywhere from 3 to 8 weeks, sometimes as long as 3 to 6 months, and then spontaneously clears. While most cases (approximately 80% 79) are first seen with this characteristic clinical pattern, there are several recognized variants. The eruption can vary markedly in its extent, showing many or few lesions. Papular, vesicular, 83,84 urticarial, or purpufic s5 lesions may occur. Papular lesions are common in blacks and in children, frequently involving the face and scalp, Occasionally the eruption is limited to one area of the body or is seen in an "inverse" distribution involving mainly the extremities or intertriginous areas 8° (Fig. 12). Le-

Journal of the American Academy of Dermatology

sions at times appear on the palms, and lesions of the mouth may be more common than we realize, because this area is often neglected during physical examination, s6 Recurrences are rare (approximately 2%). Pruritus, the main symptom associated with the rash, ranges from nonexistent to moderately severe. Mild constitutional symptoms such as malaise, pharyngitis, fever, lymphadenopathy, or headache may precede or accompany disease onset, although a study of 826 patients with PR by Bjornberg and Hellgren 78 showed that prodromal symptoms occurred with no greater frequency in patients with PR than in "healthy" controls. The histopathology of PR 77,87 shows a nonspecific subacute or chronic dermatitis and is helpful only in excluding other entities with which PR may be confused. The histopathology consists of a spongiotic dermatitis with acanthosis and focal areas of parakeratosis and a mild, perivascular lymphocytic infiltrate. The etiology of PR, while frequently debated, remains unclear. Suggested etiologies include 76 the wearing of new clothing and transmission of infective agents such as fungi, streptococci, and spirochetes. Currently, a viral agent is suspected by many to be responsible; several recent articles lend support to this theory. Although PR is generally not considered as being highly contagious, occasional concurrent or consecutive outbreaks in the same family have been reported, 77,ss McPherson et a189 found a three- to fourfold increase in the incidence of PR among dermatologists when compared with otolaryngologists. Messenger et al 9° also showed a higher incidence of PR among individuals who frequent educational establishments. These facts, along with apparent seasonal variations, lend epidemiologic support to an infective agent. Hudson et a191 searched for a causative agent by measuring acute and convalescent titers to adenovirus, influenza A and B, parainfluenza types I, II and III, and Mycoplasma, but detected no antibodies. Morgan-Capner et a192 attempted to identify an infective agent by incubating a patient's convalescent sera with an acute biopsy specimen of a PR lesion and then used fluorescein-labeled antihuman immunoglobulin to identify the presence of any circulating antibodies. None was found. Several authors 93-95report iden-

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Table II. Classification of PRP*

Type

Percentage

I

Classical adult

50

II

Atypical adult

5

III

Classical juvenile

10

IV

Circumscribed juvenile

25

V

Atypical juvenile

5

Clinical characteristics

Begins as follicular hyperkeratoses that coalesce to form sheets of erythema containing islands of normal skin; keratoderma common and has orange-yellow hue; may develop ectropion; nails may show longitudinal ridging, subungual hyperkeratosis, and splinter hemorrhages Shows variation in clinical morphology: may be ichthyosiform or eczematous may have sparse scalp hair; keratoderma is coarse and lamellated; long duration Onset during first 2 years; resembles classical adult PRP Affects prepubertal children; erythematous follicular hyperkeratosis of knees and elbows; no tendency to progress to classical PRP Onset during first few years of life; characterized by erythematous follicular hyperkeratosis; hands and feet may become scleroderrna-like; most familial cases of PRP are of this type; chronic

Prognosis: Percentage that remitin 3 years

81

20

16 32

*Modified from Griffiths WAD: Clin Exp Dermatol 5:105-112, 1980.

tifying viruslike particles in biopsy specimens of PR, suggested by some as having a structure similar to a picornavirus,93'94 and by others as similar to a togavirus. 95 Finally, a study by Takaki and Miyazaki 96identified anticytoplasmic antibodies in the sera of patients with PR, whose biopsy specimens showed a unique cytolytic degeneration of keratinocytes adjacent to Langerhans cells. Thus, evidence appears to be accumulating to support a virus as an etiologic agent of PR. Further research is needed to confirm this theory. The differential diagnosis of PR may incltlde 76'77'79 seborrheic dermatitis, tinea corporis, tinea versicolor, dermatophytid, secondary syphilis, guttate psoriasis, parapsoriasis, drug eruption, lichen planus, pityriasis lichenoides et varioliformis acuta, pityriasis alba, and autoeczematization of stasis dermatitis. Drugs that may cause a PRlike eruption include g o l d , 7~'97 bismuth, ~ metho-

promazine, 78 arsenic Lrioxide, 99 metronidazole, 99 captopril,100 tripelennamine hydrochloride,101 barbiturates, TM clonidine, ~°° and smallpox vaccination.l°a Treatment frequently is not necessary, since most eruptions are asymptomatic and covered by clothing. Reassuring the patient that the rash is not serious and that it will clear spontaneously in a few weeks is appropriate, often sufficient. However, in cases of significant pruritus or severe, generalized eruptions, topical corticosteroids and oral antihistamines may be indicated. Occasionally a short course of systemic steroids may be beneficial. 77 One of the best ways to help relieve pruritus and hasten involution of the lesions is by exposure to sunlight or ultraviolet B (UVB). Merchant and Hammond, 1°4in studying the effects of ultraviolet light on sixty-six patients with PR, found that a significant percentage (61%) improved if erythema

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was severe enough to lead to desquamation. Baden and Provan 1°5observed that lesions of PR did not occur in areas exposed to sunlight during the active phase of the disease but appeared on previously tanned areas not being further exposed. Arndt et alTM showed decreased pruritus and extent of disease in ten of twenty patients treated with UVB. its effectiveness was greatest in those patients who were treated within the first week of the eruption. PITYRIASIS RUBRA PILARIS

Pityriasis rubra pilaris (PRP) is an uncommon chronic skin disorder characterized by acuminate (tapered or pointed) follicular papules, yellowpink scaly plaques that often contain islands of normal skin, and palmoplantar keratoderma. Griffiths ~°7:°8 reported the incidence of PRP as ranging from one in 3,500 to one in 5,000 new patients. This skin disorder affects both sexes equally and has been reported in all races.~°8 Its onset can occur at any age, ~°9 but most authors t°8'lt°'m agree that the distribution curve is bimodal, with peaks in the first and fifth decades. Some cases are familial, possibly inherited as an autosomal dominant trait. ~2,113 The first case of PRP probably was described by Tarral in 1828.1°9,114 Devergie in 1857"~ described several additional cases and named the entity "pityriasis pilaris." The disease was reviewed and renamed "pityriasis rubra pilaris" by Besnier in 1889. ~16As more cases have been described over the years, different types of PRP have been recognized, leading to difficulties in classification. Most textbooks recognize an acquired and a hereditary form and whether onset is in childhood or adulthood. Other texts also delineate differences between "classical" and "atypical" PRP. Griffiths ~°~recently proposed a clinical classification that separates PRP into five different types. We believe his classification is very useful, especially as a guide for prognosis (Table II). Significant disagreement regarding the prognosis of PRP exists in the literature probably because the prognosis usually was based on whether onset was in childhood or adulthood; Overall, PRP may persist anywhere from a few months to a lifetime1°7 Juvenile types may show a tendency to relapse. Generally after 3 years, about one half of patients show complete remission. ~08.Ho

Journal of the American Academyof Dermatology The clinical hallmark of PRP is the presence of small, yellow-pink-to-red acuminate papules caused by follicular hyperkeratotic plugs (Fig. 13). Hairs may or may not be visible protruding from the keratin cones. These papules characteristically are located on the dorsal surface of the proximal phalanges in up to three fourths of patients, but they may be present in many other areas. On palpation, they are said to resemble a "nutmeg grater." The follicular papules coalesce and form erythematous, scaly plaques; one of the striking features of PRP is that these plaques frequently contain small islands of apparently normal skin. The Koebner phenomenon sometimes is present. L08 The sides and back of the neck and the extensor surfaces of the extremities often are affected, and ectropion may occur when there is heavy, waxy scaling of the face. The lesions may become generalized and progress to an erythroderma. The scalp quite often is affected, and, in fact, may be the first area involved with PRP. The scaling and erythema that are evident on the scalp can easily be misdiagnosed early in the disease course as seborrheic dermatitis. Yellow-orange hyperkeratosis of the palms and soles is another common finding (Fig. 14). This form of keratoderma can become very thick, develop painful fissures, and extend up the sides of the feet, producing a "keratodermic sandal." Nails may show subungual hyperkeratosis, longitudinal ridging, and splinter hemorrhages. 107Oral lesions may be present, consisting of a diffuse, whitish, groundglass appearance, lacy white plaques, white spots and lines, pale blue lines, and erosions) °7:17 Griffithsl07 also reported findings of eye lesions, but whether these lesions are primary or secondary to ectropion is unclear. Systemic symptoms usually are absent unless the patient is erythrodermic. Pruritus is not common, but painful fissures can develop in areas of thickening. The histologic changes in PRP, while fairly distinctive, are not diagnostic. ~Lt.jj8 The areas containing evident follicular papules show a large follicular keratin plug surrounded by perifollicular parakeratosis. Other areas show diffuse hyperkeratosis, mild irregular acanthosis, and patchy parakeratosis. A mild, chronic, perivascular infiltrate is present in the superficial dermis. There may be liquefaction degeneration of the basal layer. The

Volume 12 Number 4 April, 1985 histology often is similar to that of vitamin A deficiency and keratosis pilaris. H9 Early in the course of PRP, when only the scalp may be involved, seborrheic dermatitis is an important differential diagnostic consideration. Late in the disease, when there are widespread red, scaly plaques, psoriasis may be difficult to differentiate clinically, although the histology is quite different. Other entities in the differential diagnosis include ~°8,12°LP and LPP, phrynoderma (vitamin A deficiency), eczematous eruptions due to vitamin B deficiencies, follicular eczema, follicular ichthyosis, atypical keratosis pilaris, parakeratosis variegata, symmetric progressive erythrokeratodermia, generalized eruptive keratoacanthomas, disseminate and recurrent infundibulofolliculitis, familial dyskeratotic comedones, and drug eruptions. Upon reviewing the literature, we found approximately twenty different agents that have been used to treat PRP, many with variable or only isolated success. No single agent has been found to be consistently effective, and, since PRP may remit spontaneously, it is difficult to assess the efficacy of any of the agents used. Although the etiology of PRP is unknown, it seems to be a hyperproliferative disorder of keratinization121.122; therefore, bland emollients and keratolytic agents are helpful in treating the hyperkeratosis. Isolated reports show some success with the use of systemic vitamin C, 12~ penicillin, ~24,125isonicotinic acid hydrazide p-aminosalicylate 125,126 (Dipasic), topical aminonicotinamide 127 (although Griffiths and Ralfs ~28 recently reported failure with this substance in two patients), topical vitamin A, 129 and retinoic acid 111 (although Gunther 13° reported failure with topical retinoic acid). Oral vitamin A, 1°8'12° in doses of 150,000 to 500,000 units daily, has been used for many years and may be effective in about one third of PRP patients. While PRP resembles phrynoderma histologically, vitamin A levels in PRP patients are usually not greatly reduced. 1o9.117The efficacy of vitamin A is thought to be due to its suppression of keratinization. 12o Potential toxic side effects of hypervitaminosis A may limit its use; however, if vitamin E (1,600 IU daily) is used concurrently, TM the dosage of vitamin A may be lowered because of an apparent synergistic effect. Randle et a1132

Papulosquamous diseases 611

reported good results in six of seven patients using a short course of toxic doses of vitamin A (onehalf to one million IU/day for 5 to 14 days). Butkus 1~3 reported encouraging results using 13cis-retinoic acid (isotretinoin) to treat PRP. Goldsmith et al TM reported an overall improvement in 90% of a group of forty-five patients treated with various treatment courses of 13-cis-retinoic acid. Etretinate 13s137 is also being reported as having favorable results for treating PRP. Methotrexate, while used for treating PRP in the same manner as it has been used for treating psoriasis, 1°8 has led to improvements in only slightly more than one third of patients. Griffiths 1°8 reported successful use of azathioprine in seven of eight patients. Systemic steroids are somewhat helpful, but because of the need for continued use they are not a practical choice. Steroids are probably most beneficial in PRP patients who develop erythroderma. ~ Photochemotherapy 1°8,~35 generally has not been beneficial, and in fact some patients with PRP are photosensitive. 138 New information concerning the possible etiology of PRP has been reported. Finzi et al ~39found that the level of retinol-binding protein (RBP) was markedly reduced in eleven patients with PRP as well as in some of their relatives. The same authors also reported successfully treating three patients who had PRP and reduced RBP with stanozolol, a synthetic androgen; treatment resulted in elevation of serum RBP and improvement in cutaneous lesions.14° Stanozolol and danazol are also effective in treating hereditary angioedema, which has a Cl-esterase inhibitor protein deficiency. Finzi et a1139 similarly proposed that PRP may have a defective synthesis of RBP and that this may be a biochemical marker, probably transmitted as a mendelian-dominant gene. SEBORRHEIC DERMATITIS

Seborrheic dermatitis is a common, chronic, superficial inflammatory dermatosis showing a predilection for areas of increased sebaceous gland activity. 141-144A large percentage of the population are probably plagued by some form of seborrheic dermatitis at some time in their life. Male persons are more commonly affected than female at all ages.143 This poorly understood dermatosis appears to be associated with an oily complexion, the so-

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called "seborrheic diathesis." It may ocur in infants as early as 2 to 12 weeks, apparently due to the influence of maternal hormones, but it is seen rarely after the age of 8 to 12 months. Seborrheic dermatitis is not usually seen again until puberty. Its course is characterized by remissions and exacerbations. Involvement increases somewhat in winter m and may decrease during the summer months. 142 Symptoms of seborrheic dermatitis often are precipitated by fatigue, stress, or infection, 143The clinical expression of this condition covers a broad spectrum. Unna was the first physician to recognize the various clinical manifestations of seborrheic dermatitis and group them under the term "seborrheic eczema."~43:44 Symptoms range from mild, fluffy, white dandruff without pruritus, to excessive oiliness of the face and scalp, to highly pruritic inflammatory greasy scaling and crusting of the scalp. At times, seborrheic dermatitis may spread to the face and chest, and, in severe circumstances, becomes generalized into an exfoliative erythroderma. The incidence of seborrheic dermatitis is increased in patients with Parkinson's disease, coronary insufficiency, hypertensive cardiac failure, and in chronic alcoholics, ~43 especially those with chronic alcoholic pancreatitis. ~4s Seborrheic dermatitis-like lesions may also be seen in sprue, malabsorption disorders, epilepsy, reactions to arsenic and gold, and in obese diabetics. ~4~ A reaction to cimetidine has been reported by Kanwar et al ~46to produce a seborrheic dermatitis-like reaction. The etiology of seborrheic dermatitis remains a mystery; it generally is felt to represent a dysfunction of sebaceous gland activity. In many cases seborrheic dermatitis appears to be familial, but no pattern of inheritance has yet been determined. Various theories as to causation have been suggested over the years, including infection with yeast or bacterial organisms, emotional stress, dietary factors, hormonal stimulation, autoimmunity to epidermal scale, 142and hypersensitivity to bacteria) 43Pityrosporum ovule may be found in normal scalps but seems to be more numerous in scalps affected with seborrheic dermatitis. ~e7 Staphylococcus aureus may also be recovered more easily in patients with seborrheic dermatitis. ~4~It has not yet been shown whether the pres-

Journal of the American Academy of Dermatology ence of greater numbers of organisms in involved scalps is the cause or the effect. H6ffler et al ~48 have shown that patients with seborrheic dermatitis have a reduced number of Propionibacterium aches in the pilosebaceous ducts. Patients with seborrheic dermatitis have a reduction in the free fatty acids in their surface lipids,149 which probably is explained by a lowered concentration of propionibacterial lipase. How this might relate to the etiology of seborrheic dermatitis is still unclear. The observation that seborrheic dermatitis, like many other dermatologic conditions, is frequently exacerbated by stress is subjective and not easily proved. It has been suggested that high fat intake t4~ or increased sugar consumption ~4a m a y be contributory; a deficiency of B vitamins, such as Bj2 or biotin, has also been implicated, to2 The influence of androgenic hormones seems at least circumstantially relevant for several reasons: (1) seborrheic dermatitis is seen during infancy, presumably due to maternal hormonal stimulation, and then not again until puberty when endogenous hormone levels rise; (2) seborrheic dermatitis is more common in male persons, 14~ (3) reportedly is absent in eunuchs, and (4) frequently decreases during pregnancy. 144The hormonal influence could be due to either endogenous hormone levels or an end organ sensitivity abnormality. TM Seborrheic dermatitis may be an inflammatory hypersensitivity reaction to epidermal or bacterial antigens. 142.143These are just a few of the many proposed factors that may contribute to the etiology of seborrheic dermatitis. The classification of the different clinical variants of seborrheic dermatitis is summarized in Table III. Since the etiology of seborrheic dermatitis is poorly understood, its classification over the years has been based on the descriptive analysis of topographic variants. ~4H44 A patient may have any combination of these clinical variants at any given time. Cradle cap, a variant of seborrheic dermatitis, often begins during the third or fourth week of life as an erythematous, adherent, waxy scaling of the scalp. It has been suggested to represent a vestigial vernix caseosa, ~42,~44but more likely is due to influence of maternal hormones, c o m b i n e d with inadequate cleansing of the scalp for fear of damaging the infant's "soft spot." In most cases,

Volume 12 Number 4 April, 1985

cradle cap is easily cured by simple therapy or it clears spontaneously by 8 to 12 months of age; however, in some cases, the infant develops lesions elsewhere on the body. There may be facial involvement similar to that seen in adults, and the trunk, diaper, and flexural areas may demonstrate generally well-circumscribed yellow-red patches covered with a greasy scale (Fig. 15). Erythroderma desquamativum or Leiner's disease, a rare disorder that may occur in 2- to 4month-old infants, has been thought to represent a generalized form of seborrheic dermatitis, ~50The skin shows a generalized exfoliative dermatitis with the greasy type of scaling seen in seborrheic dermatitis. It is observed more commonly among girls and breast-fed babies of both sexes. Severe diarrhea may be present with resultant failure to thrive; infections are recurring, often due to gramnegative organisms. Before the advent of antibiotics, many infants died of this disease. A familial type of Leiner's disease has been described in the last decade ~5°'j5"~in which the fifth component of complement is functionally deficient, resulting in defective opsonization. These patients respond to antibiotics and infusions of fresh plasma or whole blood. The inheritance pattern has not yet been established, and it is unclear whether or not all cases of Leiner's disease eventually will fit into this category. Adult seborrheic dermatitis may begin as a noninflammatory, dry, flaky desquamation of the scalp referred to as "pityriasis sicca" or common dandruff. This is the extent of the disease in some persons; in many, however, it progresses to a pruritic, inflammatory dermatitis of the scalp with erythema, greasy scaling, crusts, pustules, and even papulovesicles, occasionally referred to as "pityriasis steatoides." The entire scalp may be involved, with extension onto nonhairy adjacent areas such as the postauricular area and the forehead, where it is called the "corona seborrheica." Some authors ~4L,15~ believe that seborrheic dermatitis may contribute to male-pattern baldness, but definite proof is lacking. Facial seborrheic dermatitis may be preceded by an oily complexion referred to as "seborrhea oleosa," the so-called "seborrheic diathesis." The eyebrows may show erythema, scaling, and crusting. Involvement of the nasolabial fold is common and may aid in the

Papulosquamous diseases 613

Table III. Clinical variants of seborrheic dermatitis I. Seborrheic dermatitis of infancy A. Cradle cap B. Body lesions: flexural, diaper, and trunk areas C. Leiner's disease 1. Nonfamilial 2. Familial C5 dysfunction II. Seborrheic dermatitis of adults A. Scalp seborrheic dermatitis 1. Pityriasis sicca (dandruff without inflammation) 2. Inflammatory--including extension onto nonhairy adjacent areas: corona seborrheica, postauricular B, Facial seborrheic dermatitis 1. Pityriasis oleosa--the "seborrheic diathesis": oily skin 2. Eyebrows 3. Marginal blepharitis, conjunctivitis 4. Nasolabial fold 5. Beard involvement C, Otitis externa--seborrheie dermatitis is probably the most common cause D. Flexural (intertriginous) seborrheic dermatitis: axillary, inframammary, umbilical, intergluteal, and groin areas E. ]Yuncal seborrheic dermatitis 1. Petaloid 2. Pityriasiform F. Generalized seborrheic dermatitis: Can progress to an exfoliative erythroderma

diagnosis of seborrheic dermatitis. This area may also show erythema, scaling, and crusting and is frequently pruritic. Men's beards may be affected; lesions are similar to those found in the scalp, except that they tend to be more follicular in location and show more pustulation. 142.143 Seborrheic blepharitis, or "marginal blepharitis," is an erythematous, scaly, granular inflammation of the eyelid margin. The shedding of epithelial debris into the eye m a y produce a conjunctivitis. ~54 Although frequently unrecognized, seborrheic dermatitis is probably the most common cause of otitis externa.144 There is pruritic, erythematous scaling of the external auditory meatus with extension onto the concha. Infectious eczematoid dermatitis of the ear may occur due to bacterial superinfection. Flexural involvement of the axillae usually begins in the apices and at presentation has the same

614 Fox and Odom

yellowish-red patches with greasy scaling. Inframammary, umbilical, intergluteal, and groin lesions may be clinically indistinguishable from intertrigo, and, because of the occlusive nature of these areas, secondary infection with Candida, Staphylococcus aureus, Streptococcus, and dermatophytes is common. Two types of truncal lesions may occur in seborrheic dermatitis. The "petaloid" type is common on the chest and interscapular area and begins as follicular scaly papules that become confluent to form circinate patterns like the "petals" of a flower. The "pityriasiform" type of seborrheic dermatitis consists of oval papulosquamous lesions resembling PR. In severe cases, generalized seborrheic dermatitis may result in an exfoliative erythroderma. The diagnosis of seborrheic dermatitis usually is made clinically, based on the distribution and morphology of lesions. It is sometimes difficult to differentiate this entity from other disease entities. Psoriasis and seborrheic dermatitis at times seem to overlap, leading to use of the terms "seborrhiasis" and "sebopsoriasis. ''~5° Infantile seborrheic dermatitis of the diaper area may be difficult to differentiate from psoriasis; Thomsen ~55 believes this type of "napkin dermatitis" may, in fact, be an early form of psoriasis. Other diseases to be considered in the differential diagnosis of seborrheic dermatitis in infancy include142,144,15°Letterer-Siwe disease, atopic dermatitis, tinea capitis, pediculosis capitis, irritant diaper dermatitis, monilial diaper dermatitis, pityriasis rubra pilaris, Darier's disease, chronic granulomatous disease of childhood, acrodermatitis enteropathica, and the Wiskott-Aldrich syndrome. In adults, the following entities also have to be consideredm.~44: contact dermatitis, intertrigo, PR, pemphigus erythematosus, familial benign pemphigus, lupus erythematosus, tinea versicolor, erythrasrna, and drug eruptions. Ferr~indiz et aP56 recently reiterated that an acrodermatitis enteropathica-like syndrome can develop in patients on total parenteral alimentation because of an acquired zinc deficiency. The histopathology of seborrheic dermatitis is nonspecific and rarely is obtained for diagnostic purposes. Histopathology can show an acute, subacute, or chronic dermatitis. 157Acute lesions may

Journal of the American Academy of Dermatology

show edema of the papillary dermis with focal spongiosis, a crust containing neutrophils, superficial telangiectases, and a scant superficial perivascular infiltrate containing mononuclear cells and neutrophils. Subacute lesions differ by lacking neutrophils in the perivascular infiltrate and by development of acanthosis. Chronic lesions lack spongiosis and neutrophil-containing crusts, show more of a psoriasiform epidermal hyperplasia with focal parakeratosis, and contain numerous telangiectases and fewer inflammatory cells in the dermis. According to Ackerman, ~57 simple dandruff does not have spongiosis or inflammation, but merely shows areas of orthokeratosis and parakeratosis. Cradle cap is the easiest form of seborrheic dermatitis to treat, probably because it is a temporary process. Gentle debridement of the scalp scaling can be accomplished by using a mild keratolytic shampoo containing sulfur or salicylic acid and a soft cosmetic brush or rough washcloth. The parents should be reassured that they will not harm the infant's fontanel. Body lesions usually respond promptly to topical nonfluorinated corticosteroids, Infantile seborrheic dermatitis has been thought by some to represent a deficiency of biotin or essential fatty acids; however, a recent study by Erlichman et al ~8 found that biotin therapy was not beneficial and that affected infants did not have a measurable deficiency of linoleic acid. Superinfection and bacteria in Leiner's disease must be treated with appropriate antibiotics, and dehydration must be corrected. The skin may be treated with keratolytics and topical corticosteroids. Familial Leiner's disease with a documented dysfunctional C5 should be treated with infusions of fresh plasma or whole blood. The treatment of adult seborrheic dermatitis recently was summarized by Cram,* Richardson, ~9 and Ellis and Stawiski. ~ Therapy for the scalp consists of antiseborrheic shampoos containing selenium sulfide, zinc pyrithione, salicylic acid and sulfur, chloroxine, or tar. In pruritic or inflammatory cases, topical corticosteroid lotions or sprays applied to the scalp are beneficial. For removing thick crusts in the scalp, an overnight ap*Cram DL: How I treat seborrheic dermatitis. Med Times 108:7476, 1980.

Volume 12 Number4 April, 1985 plication of a keratolytic agent, such as Baker's P & S liquid or Pragmatar, is helpful. For nonhairy areas, topical corticosteroid creams are usually effective. Because of the chronicity of this disease, potent fluorinated steroids that produce a higher incidence of undesirable side effects when used for prolonged periods should be avoided. When secondary yeast or bacterial infection is suspected, a topical preparation containing iodochlorhydroxyquin may be beneficial. Solano ~1 reported that topical application of the nonsteroidal antiinflammatory agent, hydroxamic acid (bufexamac), was effective in treating seborrheic dermatitis. Patients with Parkinson's disease often show partial resolution of their seborrheic dermatitis while they are taking levodopa. 162"164External otitis due to seborrheic dermatitis can be treated with corticosteroid solutions. It is probably well to avoid long-term administration of neomycin-containing products to avoid possible sensitization. Storrs 16"~reported successfully treating seborrheic otitis with a suspension of human cerumen. Finally, seborrheic blepharitis may be controlled with daily debridement of the eyelid margin, using a cotton-tipped applicator dipped in baby shampoo. Topical antibiotic ointments are helpful in stubborn cases. If corticosteroids are used to treat the blepharitis, the patient should probably be followed by an ophthalmologist, as steroid preparations used in the eye may produce increased intraocular pressure, glaucoma, activation of a latent herpetic infection, and cataracts. GIANOTTI-CROSTI SYNDROME Papular acrodermatitis of childhood (PAC, infantile papular acrodermatitis, Gianotti-Crosti syndrome) is a cutaneous manifestation of hepatitis B infection occurring in children age 3 months to 15 years of age, with a peak incidence from 1 to 6 years. 166A few cases have been observed in young adults. ~67 Boys and girls are affected equally, ~8 and there is no apparent seasonal variation in incidence. 169 Children with Down's syndrome are more susceptible than others. 169 First recognized by Gianotti in 1955,17° PAC was further described by Crosti and Gianotti in 1957.171 The cutaneous eruption, which is fairly distinctive and occurs abruptly in an apparently healthy child,

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consists of nonpruritic, flat, monomorphous, erythematous, lichenoid papules that erupt symmetrically on the face, buttocks, and extensor aspect of the extremities (Fig. 16). In older children, papules are small (1-2 ram), whereas in infants they may be as large as 5 ram. ~69The lesions may exhibit a Koebner phenomenon and may become purpuric and scaly. I~s'169 Mucous membrane lesions have not been reported. 169 The rash develops over a period of a few days and usually lasts 15 to 20 days or longer. Occasionally a transient rash is present on the trunk at the onset. Systemic symptoms, if present, usually are mild and consist of malaise, a low-grade fever, and, at times, diarrhea, A generalized lymphadenopathy, most commonly noted in the axillary and inguinal areas, persists for 2 to 3 months.~69 Splenomegaly occasionally is present but tends not to persist. Laboratory values may reveal a leukopenia with a relative increase in monocytes and a mild hypochromic anemia. In 1964, Crosti and Gianotti 172 reported that most cases of PAC were associated with anicteric hepatitis, and in 1970 hepatitis B surface antigen (HBsAg) was detected in the blood of patients with PAC. 173 The hepatitis may begin with a rash, or the rash may appear 1 to 2 weeks later and is characterized by nontender hepatomegaly that lasts 1 to 2 months. The aspartate transaminase (AST; SGOT), alanine transaminase (ALT; SGPT), aldolase, lactic dehydrogenase, alkaline phosphatase, and sulfobromophthalein sodium (Bromsulphalein) retention levels are all elevated for 1 to 3 months, but serum bilirubin remains normal, except in the less common, icteric cases. 169 Liver biopsy shows features consistent with an acute viral hepatitis. ,74 The hepatitis usually is benign and self-limiting but may result in chronic persistent hepatitis, or, rarely, chronic periportal aggressive hepatitis. '69,175 Subtyping of the HBsAg shows a preponderance of the determinant ayw174:76; a few cases have been reported to be adr 176 or adw.177 The subtype ayw is common in the Mediterranean area, where PAC was first described; in Japan, where there was an epidemic of PAC,176ayw subtype is rare, but it was found to be present in most children with PAC. HBsAg is usually detectable about 10 days following onset of the rash~66; in most cases it decreases in a few months, but it

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Fig. 18. EPS in a young child. Note the symmetry of the lesions. Table IV. Classification of papular acrodermatitis I.Gianotti-Crosti syndrome (PAC)--associated with hepatitis B II. Papulovesicular acrolocated syndrome (PAS) A. Associated with hepatitis other than type B 1. Known etiology, e.g., Epstein-Barr virus 2. Idiopathic B. Not associated with hepatitis 1. Known etiology, e.g., coxsackie A-16, parainfluenza virus 2. Idiopathic

may persist for months or years. 169 Antihepatitis B core antibody (anti-HBc) also becomes positive soon after the dermatitis appears and persists for 3 to 5 months. 174 When patients become HBsAgnegative, antihepatitis B surface antibody (antiHBs) can be detected, with a peak at 6 to 12 months after PAC. 169 Hematoxylin and eosin staining of a biopsy specimen of PAC is nonspecific and reveals a lymphohistiocytic infiltrate in the superficial dermis, concentrated around capillaries, which may show endothelial swelling. ~69 There may be acanthosis and focal spongiosis and parakeratosis. ~78 Direct immunofluorescent studies of PAC have been negative for immunoglobulins and fibrinogen, but all three adults described by Claudy et a1167 had de-

posits of C3 in the dermal vessels. Attempts to identify HBsAg, HBcAg, or HBeAg in biopsy specimens of skin have failed to reveal their presence. 167.179.18oGianotti believes that lack of antigens and antibodies in the skin lesions of PAC lends evidence against vasculitis as the etiology of the skin eruption. ~8° The clinical eruption of PAC is often distinctive, but the differential diagnosis may include papular PR, LP, Letterer-Siwe disease, lichenoid drug eruption, lichen urticatus, and LN. Probably the most important consideration in the differential diagnosis is the papulovesicular acrolocated syndrome (PAS), t69 This is Gianotti's designation of disorders similar to PAC that also affect children but are not associated with hepatitis or hepatitis B virus. The lesions of PAS tend to be pruritic, polymorphic, more often vesicular or edematous, and may last longer than in PAC. Many of these cases have been idiopathic, whereas others have been shown to be cutaneous manifestations of infectious mononucleosis, the late stage of Henoch-Sch6nlein purpura, and vaccinids (due to hematogenous diffusion of the vaccinia virus)169 Konno et a1181described a PAC-like eruption and hepatitis in three patients whose test results were negative for HBsAg but positive for antibodies to Epstein-Barr virus. James et a1182 described a patient with a PAC-like eruption without hepatitis

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Table Vo Papulosquamous drug eruptions I. Psoriasiform eruptions Amodiaquine Oxprenolol Chloroquine Pindolol Debrisoquin Practolol Lithium Propranolol Mepacrine Sulfonamides II. Lichenoid and lichen planus-like eruptions Amiphenazole Para-aminosalicylic acid Benzothiadiazines Paraphenylenediamine Bismuth salts Captropril Penicillamine Carbamazepine Phenothiazines Chlordiazepoxide Pindolol Chloroquine Practolol Chlorpropamide Propranolol Dapsone Pyritinol Ethambutol Quinacrine Furosemide Quinidine Gold salts Quinine Hydroxychloroquine Streptomycin Labetalol Sulfonylureas Levamisole Tetracyclines Mepacrine Thiazides Meprobamate Triprolidine Methyldopa III. Pityriasis rosea-like eruptions Arsenic trioxide Methopromazine Barbiturates Metoprolol Bismuth Metronidazole Captopril Smallpox vaccination Clonidine Tripelennamine hydroGold salts chloride IV. Seborrheic dermatitis-like eruptions Arsenic Gold salts Cimetidine Methyldopa

w h o was found to have positive viral cultures and titers to coxsackie virus A-16. Rubenstein et a1183 reported two cases of patients with PAC-like dermatitis associated with hepatitis, but whose test results were negative for HBsAg. In a recent review, Spear and Winkelmann ~84reported a case of infantile acrodermatitis that clinically resembled PAC but that revealed a parainfluenza virus from a throat culture. Obviously, confusion exists in the literature about classification of these disorders. Gianotti distinguishes three main types of PAS: types A, B, and C, based on their clinical morphology~69; however, Spear and Winkelmann ~84 pointed out that this distinction may not always be possible. Joaquin and Marks 18~proposed referring

Fig. 19. Psoriasiform dermatitis secondary to lithium administration. to PAC with hepatitis B as"Gianotti disease," and other types of PAC-like eruptions as "GianottiCrosti syndrome." We feel this terminology would only further confuse an already muddled classification. We prefer to think of Gianotti-Crosti syndrome (or PAC) as the eruption typically associated with hepatitis B. This eponym is already well established in major textbooks. PAC-like eruptions could then be referred to as PAS, with hepatitis due to another agent such as Epstein-Barr virus or undetermined etiology; or without evidence of hepatitis, either idiopathic or due to an identified agent such as coxsackie A-16 or parainfluenza virus (Table IV). As these diseases are more thoroughly studied, other etiologic agents no doubt will be identified, and the classification can then easily be expanded. Since PAC is self-limited and usually asymptomatic, no specific therapy is indicated. In fact, Hjorth et al ~86reported that topical corticosteroids

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Fig. 20. Lichenoid eruption due to gold therapy.

may actually have an adverse effect on the course of the skin eruption. The most important factor is to measure the liver function tests and determine if the patient is HBsAg-positive. Although PAC usually is not highly infectious, i69 an epidemic did occur in Japan. 176 A follow-up study of this epidemic by Toda et al ~87 shows that parents, especially mothers, may contract hepatitis B from their children with PAC. Parental risk is even greater when children have PAC at 1 year of age or younger, because this age group tends to have a higher incidence of persistent HBs antigenemia. OTHER PAPULOSQUAMOUS CONDITIONS

Many other derrnatologic conditions may become papulosquamous at some time during their course, Many of these conditions are classified under different groups, based on other important characteristics, such as pigmented purpuras, genodermatoses, photosensitivities, dermatophytoses, treponemal infections, parasitic infestations, nutritional deficiencies, and drug eruptions. Comments on a few of these entities seem appropriate in this review. Lichen aureus has been considered a papulosquamous eruption, but we will explain why it belongs with the pigmented purpuric dermatoses. An entity called erythrokeratodermia progressiva symmetrica is briefly discussed because it is not well known. A few corn-

ments will also be made concerning drag-related eruptions. "Lichen purpuricus" was first described in the British literature in 1958 by Martin. 188 Calnan 189 in 1960 described a similar case which he termed "lichen aureus." In the American literature, Farrington 19° reported a case in 1970. This entity is characterized by sudden appearance of a circumscribed group of lichenoid papules and plaques ranging in color from golden-rust ("aureus") to dark purple ("purpuricus") (Fig. 17). There is usually no history of trauma or preceding systemic medication. Although lichen aureus may appear on any part of the body, its usual location is on the arms or legs. Persons of both sexes and any age can be affected. One case showing Koebner phenomenon has been reported. TM Initially, prutitus may be present, after which the lesion tends to persist unchanged indefinitely, regardless of type o f treatment. Reinhardt et a] 191 reported temporary improvement in a lesion of lichen aureus duting treatment for a urinary tract infection with ampicillin. Rudolph t92 observed rapid improvement in one case of lichen aureus following treatment with topical halcinonide cream. Histologically, ~93-~95,* there may be epidermal *Wilson RK, Picou KA, Babcock WS: Lichen aureus: Report of a case occurring on the ann. I Assoc Milit Dermatol 7:40-41, 1981,

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thinning and minimal hyperkeratosis; often, however, there are no epidermal findings. A small grenz zone separates the epidermis from an upper dermal bandlike infiltrate of lymphocytes and histiocytes. Some biopsy specimens show capillaries with prominent endothelial cells and thickening of cell walls, perivascular lymphohistiocytic infiltrate, and extravasation of erythrocytes. Abundant hemosiderin deposits can be seen in the histiocytes by iron stains. Lichen aureus is a rare entity; only a few scattered cases have been reported in the past 20 years. In the past, this ill-defined entity has been classified with papulosquamous or lichenoid disorders, or, because of its rarity, has been omitted from some texts. Recently several authors have proposed that lichen aureus is a type of pigmented purpuric dermatosis, 19H93 perhaps a variant of lichenoid purpura of Gougerot and Blum. 196We COrlcur with this classification and have, therefore, excluded lichen aureus as a major papulosquamous disorder. Erythrokeratodermia progressiva symmetrica (EPS) is a rare erythematous papulosquamous disorder that appears to be inherited in an autosomal dominant pattern. 197The first case was reported by Gottr0n in 1922. j98 Ruiz-Maldonado and coworkers ~97 in 1982 reviewed the entity and presented ten cases. To date, approximately twentytwo cases have been reported in the literature. The EPS lesions usually occur during the first year of life, progress for 1 or 2 years, and tend to remain unchanged thereafter. Partial regression may occur after puberty. The lesions are well defined, erythematosquamous plaques having an uncanny characteristic symmetry (Fig. 18). Some lesions may be dyschromic. The head, extremities, and buttocks are mainly affected with relative sparing of the chest and abdomen. Palms and soles may be involved. Lesions generally are nonpruritic. The histopathology of EPS is similar to that of psoriasis. Some differences are that EPS has less suprapapillary thinning, more focal parakeratosis, no Munro microabscesses, and presence of a granular layer. Differential diagnosis of this distinctive genodermatosis may include psoriasis, chronic dermatitis, verrucous epidermal nevus, X-linked

Papulosquamous diseases 619

recessive ichthyosis, ichthyosis vulgaris, erythrokeratodermia variabilis, and bullous congenital ichthyosiform erythroderma. Ruiz-Maldonado et al m97 reported partial improvement following topical coal tar therapy and complete remission using maintenance oral aromatic retinoids. Finally, a wide variety of drugs can produce papulosquamous eruptions (Table V) (Figs. 19 and 20). (Many of the agents are discussed under the particular type of papulosquamous eruption that they may simulate.) The importance of reviewing the patient' s drug history when considering the diagnosis of any cutaneous eruption cannot be overemphasized. REFERENCES

Psoriasis 1. Cram DL: Psoriasis: Current advances in etiology and treatment. J AM ACA~ DERMATOL4:1-14, 1981.

Parapsoriasis 2. Lambert WC, Everett MA: The nosology of parapsoriasis. J AM ACADDERMATOL5:373-395, 1981.

Lichen planus 3. Amdt KA: Lichen pl anus, in Fitzpatrick TB, Eisen AZ, Wolff K, et al, editors: Dermatology in general medicine, ed. 2. New York, 1979, McGraw-Hill Book Co., pp. 655-661. 4. Demis DJ, Dobson RL, McGuire J: Clinical dermatology. Hagerstown, 1979, Harper & Row, Publishers Inc., vol 1. unit 1-9, pp. 1-12. 5. Rook A, Wilkinson DS, Ebling FJG: Textbook of dermatology, ed. 3. Oxford, 1979, Blackwell Scientific Publications, pp. 1483-1496. 6. Moschella SL, Pillsbury DM, Hurley JH: Dermatology. Philadelphia, 1975, W. B. Saunders Co,, pp. 434-442. 7. Calnan C, Meara RH: St. John's hospital diagnostic index. Trans St. Johns Hosp Dermatol Soc 39:56, 1957. 8. Schmidt H: Frequency, duration and localization of lichen planus. Acta Derm Venereol (Stockh) 41:164, 1961. 9. Wilson E: On lichen planus. J Cutan Med 8:117, 1869. 10. Walker DM: Identification of subpopulations of lymphocytes and macrophages in the infiltrate of lichen planus lesions of skin and oral mucosa. Br J Dermatol 94:529-534, 1976. 11. Brody I: Electron-microscopic demonstration of bacteria in the skin of patients with lichen tuber planus. Nature 207:96-98, 1965. 12. Baart de la Faille-Kuyper EH, Baart de la Faille H: An immunof/uorescence study of lichen planus. Br J Dermatol 90:365-371, 1974. 13. Dahl MV: Clinical immunodermatology. Chicago, 1981, Year Book Medical Publishers Inc., pp. 256-257. 14. Saurat JH, Didier I, Gluckman E, et al: Graft-versus-

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host reaction and lichen planus-like eruption in man. Br J Derrnatol 92:591-592, 1975. Saurat JH, Gluckman E, Bussel A, et al: The lichen planus-like eruption after bone marrow transplantation. Br J Dermatol 92:675-681, 1975. James WD, Odom RB: Graft-v-host disease. Arch Dermatol 119:683-689, 1983. Domonkos AN, Arnold HL, Odom RB: Andrew's Diseases of the skin. Philadelphia, 1982, W. B. Saunders Co., pp. 260-274. Copeman PWN, Tan RSH, Timlin D, et al: Familial lichen planus. Br J Dermatol 98:573-577, 1978. Veien NK, Risum G, Paulli Jorgensen H, et al: HLA antigens in patients with lichen planus. Acta Derm Venereol (Stockh) 59:205-209, 1979. Mahood JM: Familial lichen planus. Arch Dermatol 119:292-294, 1983. Bazemore JM, Johnson HH, Swanson ER, et al: Relationship of quinaerine hydrochloride to lichenoid dermatitis. Arch Dermatol Syph 54:308-324, 1946. Buckley WR: Liehenoid eruptions following contact dermatitis. Arch Dermatol 78:454-457, 1958. Seehafer JR, Rogers RS III, Fleming CR, et al: Lichen planus-like lesions caused by penicillamine in primary biliary cirrhosis. Arch Dermatol 117:140-142, 1981. Levy RS, Fisher M, Alter JN: Penicillamine: Review and cutaneous manifestations. J AM ACADDERMATOL 8:548-558, 1983. Bravard P, Hannedouche T, Thomine E, et al: Eruption lich6noide induite par le eaptopril. Presse Med 12: 577-578, 1983. Dusek JJ, Frick WG: Lichen planus: Oral manifestations and suggested treatments. J Oral Maxillofac Surg 40:240-244, 1982. Smith MJA: Oral lichen planus and diabetes mellitus: A possible association. J Oral Meal 32:110-112, 1977. Alinoir A, Barella PA, Benoldi D: Erosive lichen planus involving the glans penis alone. Int J Dermatol 22:3738, 1983. Norton LA: Nail disorders: A review. J AM ACADDERMATOL2:451-467, 1980. Scher RK, Fischbein R, Ackerman AB: Twenty-nail dystrophy: A variant of lichen planus. Arch Dermatol 114:612-613, 1978. Mora RG, Nesbitt LT Jr, Brantley JB: Lichen planus pemphigoides: Clinical and immunofluorescent findings in four cases. J AM ACADDERMATOL8:331-336, 1983. Anderson RL, Cullen SI: Pseudopelade of Brocq secondary to lichen planus. Cutis 17:916-918, 1976. Horn RT, Goette DK, Odom RB, et al: Immunofluorescent findings and clinical overlap in two cases of follicular lichen planus. J AM ACADDERMATOL7:203207, 1982. Cram DL, Kierland RR, Winkelmann RK: Ulcerative lichen planus of the feet. Arch Dermatol 93:692-701, 1966. Crotty CP, Su WPD, Winkelmann RK: Ulcerative lichen planus: Follow-up of surgical excision and grafting. Arch Dermatol 116:1252-1256, 1980. Lever WF, Sehaumburg-Lever G: Histopathology of the skin, ed. 6. Philadelphia, 1983, J. B. Lippineott Co., pp. 151-156. Sehgal VN, Abraham GJS, Malik GB: Griseofulvin

therapy in lichen planus: A double-blind controlled trial, Br J Dermatol 87:383-385, 1972. 38. Thomas JR HI, Doyle JA: The therapeutic uses of topical vitamin A acid. J AM AChDDERMATOL4:505-513, 1981. 39. Ortonne JP, Schmitt D, Alario A, et al: Oral photochemotherapy in lichen planus (LP) and mycosis fungoides (MF): Ultrastructural modifications of the infiltrating cells. Acta Derm Venereol (Stockh) 59:211-218, 1979. 40. Mahrle G, Meyer-Hamme S, Ippen H: Oral treatment of keratinizing disorders of skin and mucous membranes with etretinate: Comparative study of 113 patients. Arch Dermatol 118:97-100, 1982. Lichen nitidus

41. Pinkus F; Uber eine neue kn6tchenf6rmige Hautemption: Lichen nitidus. Arch Dermatol 85:11-36, 1907. 42. Lapins NA, Willoughby C, Helwig EB: Lichen nitidus. A study of forty-three cases. Cutis 21:634-637, 1978. 43. Domonkos AN, Arnold HL, Odom RB: Andrew's Diseases of the skin. Philadelphia, 1982, W. B. Saunders Co., pp. 269-271. 44. Weiss RM, Cohen AD: Lichen nitidus of the palms and soles. Arch Dermatol 104:538, 1971. 45. Buono PL, Kurtin SB: Lichen nitidus of the palms. Cutis 22:74-76, 1978. 46. Krook G: Purpura in lichen nitidus generalisatus. Acta Derm Venereol (Stockh) 39:238, 1959. 47. Amdt KA: Lichen nitidus, in Fitzpatrick TB, Eisen AZ, Wolff K, et al, editors: Dermatology in general medicine, ed. 2. New York, 1979, McGraw-Hill Book Co., pp. 661-665. 48. Hurwitz S: Clinical pediatric dermatology. Philadelphia, 1981, W. B. Saunders Co., pp. 100-101. 49. Rook A, Wilkinson DS, Ebling FJG: Textbook of dermatology, ed. 3. Oxford, 1979, Blackwell Scientific Publications, pp. 1498-1500. 50. Wilson HTH, Bett DCG: Miliary lesions in lichen planus. Arch Dermatol 83:920-923, 1961. 51. Kint A, Meysman L, Bugingo G, et al: Lichen nifidus and Crohn's disease. Dermatologica 164:272-277, 1982. 52. Sordet M, Chavaz P: Trois cas de lichen nitidus chez des patients de race noire. Dermatologica 162:455-46i, 1981. 53. Jetton RL, Eby CS, Freeman RG: Vesicular and hemorrhagic lichen nitidus. Arch Dermatol 105:430-431, 1972. 54. Miller RAW: The Koebner phenomenon. Int J Dermatol 21:192-197, 1982. 55. Ackerman AB: Histologic diagnosis of inflammatory skin disease. Philadelphia, 1978, Lea & Febiger, pp. 412-414. 56. Bardaeh H: Perforating lichen nitidus. J Cutan Pathol 8:111-116, 1981. 571 Clausen J, Jacobsen FK, Brandup F: Lichen nitidus: Electron microscopic and immunofluorescence studies, Acta Derm Venereol (Stockh) 62:15-19, 1982. 58. Dahl MV: Clinical immunodermatology. Chicago, 1981, Year Book Medical Publishers Inc., pp. 256-257. 59. Waisman M, Dund0n BC, Michel B: Immunofluores-

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cent studies in lichen nitidus. Arch Dermatol 107:200203, 1973. Lichen striatus

60. Staricco RG: Lichen striatus. Arch Dermatol 79:311324, 1959. 61. Hurwitz S: Clinical pediatric dermatology. Philadelphia, 1981, W. B. Saunders Co., pp. 55-56. 62. Rook A, Wilkinson DS, Ebling FJG: Textbook of dermatology, ed. 3. Oxford, 1979, Blaekwell Scientific Publications, pp. 347-348. 63. Senear FE, Caro MR: Lichen striatus. Arch Derrnatol Syph 33:116-133, 1941. 64. Demis DJ, Dobson ILL, McGuire J: Clinical dermatology. /iagerstown, 1979, Harper & Row, Publishers Inc., vol 1, unit 1-11, pp. 1-2. 65. Mopper C, Horwitz DC: Bilateral lichen striatus. Cutis 8:140-141, 1971. 66. Owens DW: Lichen striatus with onychodystrophy. Arch Dermatol 105"457-458, 1972. (Letter to Editor.) 67. Kaufman JP: Lichen striatus with nail involvement. Cutis 14:232-234, 1974. 68. Meyers M, Storino W, Barsky S: Lichen striatus with nail dystrophy. Arch Dermatol 114:964-965, 1978. (Letter to Editor.) 69. Baran R, Dupr6 A, Lauret P, et ah Le lichen striatus onychodystrophique. Ann Dermatol Venereol 106:885891, 1979. 70. Domonkos AN, Arnold IlL, Odom RB: Andrew's Diseases of the skin. Philadelphia, 1982, W. B. Saunders Co., p. 271. 71. Reed RJ, Meek T, Ichinose H: Lichen striatus: A model for the histologic spectrum of lichenoid reactions. J Cutan Pathol 2:1-18, 1975. 72. Pink:us F: Ueber eine besondere Form strichfrrmiger HautaussehRige. Dermatol Ztschr 11:19-27, 1904. 73. Stewart WM, Lauret P, Pietrini P, et ah Lichen striatus: crit~res histologiques. Ann Dermatol Venereol 104:132135, 1977. 74. LeverWF, Schaumhurg-Lever G: Histopathotogy of the skin, ed. 6. Philadelphia, 1983, J. B. Lippincott Co., p. 158. 75. Charles CR, Johnson BL, Robinson TA: Lichen striatus: A clinical, histologic and electron microscopic study of an unusual case. J Cutan Pathol 1:265-274, 1974. Pityriasis rosea 76, Rook A, Wilkinson DS, Ebling FJG: Textbook of dermatology, ed. 3. Oxford, 1979, Blackwell Scientific Publications, pp. 673-676. 77. Domonkos AN, Arnold HL, Odom RB: Andrew's Diseases of the skin. Philadelphia, 1982, W. B. Saunders Co., pp. 252-254. 78. Bjornberg A, Hellgren L: Pityriasis rosea: A statistical, clinical and laboratory investigation of 826 patients and matched healthy controls. Acta Derm Venereol (Stockh) 42(suppl 50):1-68, 1962. 79. Hendricks AA, Lohr JA: Pityriasis rosen in infancy. Arch Dermatol 115:896-897, 1979. 80. Demis DJ, Dobson RL, McGuire J: Clinical dermatology. Hagerstown, 1979, Harper & Row, Publishers Inc., vol 1, unit 1-14, pp. 1-6.

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81. Gibert CM: Trait6 pratique des maladies speciales de la peau et de la syphilis, ed. 3. Paris, 1860, H. Plon. 82. Percival G/h Pityriasis rosea. Br J Dermatol 44:241253, 1932. 83. Baker R J, Johnson WC: Vesicular pityriasis rosen. Curls 8:341-342, 1971. 84. Garcia RL: Vesicular pityriasis rosea. Arch Dermatol 112:410, 1976. 85. Verbov J: Purpuric pityfiasis rosea. Dermatologica 160:142-144, 1980. 86. Dupr6 A, Lassere J, Christol B, et al: Pityriasis ros6 de Gibert avec localisatiott endo-buecale. Ann Dermatol Venereol 104:873-875, 1977. 87. Lever WF, Schaumburg-Lever G: Histopathology of the skin, ed. 6. Philadelphia, 1983, J. B. Lippincott Co., p. 150. 88. Davies SWV: Case clustenng in pityriasis rosea: Support for role of an infective agent. Br Med J 284:I478, 1982. (Letter to Editor.) 89. McPherson A, McPherson K, Ryan T: Is pityriasis rosea an infectious disease? Lancet 2:1077, 1980. (Letter to Editor.) 90, Messenger AE, Knox EG, Summerly R, et al: Case clustering in pityriasis rosea: Support for role of an infective agent. Br Med J 284:371-373, 1982. 91. /iudson LD, Adelman S, Lewis CW: Pityriasis rosea. J AM ACADDERMATOL4:544-546, 1981. 92. Morgan-Capner P, Hodgson J, Pattison J, et al: Case clustering in pityriasis rosea: Support for role of an infective agent. Br Med J 284:977, 1982. (Letter to Editor.) 93. Raskin J: Possible dermatropic virus associated with pityriasis rosea. Acta Derm Venereol (Stockh) 48:474481, 1968. 94. Metz J: An electron microscopic investigation of pityriasis rosea. J Cutan Pathol 4:228, 1977. (Abst.) 95. Aoshima T, Komara J, Ofuji S: Virus-like particles in the herald patch of pityriasis rosea. Dermatologiea 162:64-65, 1981, (Letter to Editor.) 96. Takaki Y, Miyazaki H: Cytologic degeneration of keratinocytes adjacent to Langerhans cells in pityfiasis tosea (Gibert). Acta Derm Venereo! (Stockh) 56;99-103, 1976. 97. Wile UJ, Courville CJ: Pityriasis rosea-like dermatitis following gold therapy. Arch Dermatol 42:1105-1112, 1940. 98. Dobes WL, Alden HS: Pityriasis rosea-like drug eruptions due to bismuth. South Med J 42:572-578, 1949. 99. Maize JC, Tomecki KJ: Pityriasis roseaqike drug eruption secondary to metronidazole. Arch Dermatol 113: 1457-1458, 1977. (Letter to Editor.) 100. Wilkin JK, K~rkendall WM: Pityriasis roseaqike rash from captopril. Arch Dermatol 118:186-187, 1982. 101. Epstein E: Dermatitis occurring during therapy with tripelennamine hydrochloride ("pyribenzamine hydrochloride"). JAMA 134:782, 1947. 102. Beerrnan H, Kirshbaum BA: Drug eruptions (dermatitis medicamentosa), in Moschella SL, Pillsbury DM, Hurley HJ, editors: Dermatology. Philadelphia, 1975, W. B. Saunders Co,, p. 365. 103. Witherspoon FG, Thibodeaux DJ: Pityriasis rosea-like eruption following smallpox vaccination. Arch Dermatol 76:109-110, 1957.

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104. Merchant M, Hammond R: Controlled study of ultraviolet light for pityriasis msea. Cutis 14,548-549, 1974. 105. Baden HP, Provan J: Sunlight and pityriasis rosea. Arch Dermatol 113:377-378, 1977. (Letter to Editor.) 106. Arndt KA, Paul BS, Stem RS, et al: Treatment of pityriasis msea with UV radiation. Arch Dermatol 119:381-382, 1983.

Pityriasis rubra pilaris 107. Griffiths WAD: Pityriasis rubra pilaris--an historical approach, II. Clinical features. Clin Exp Dermatol 1:3750, 1976. 108. Griffiths WAD: Pityriasis rubra pilaris. Clin Exp Dermatol 5:105-112, 1980. 109. Gross DA, Landau JW, Newcomer VD: Pityriasis mbra pilaris: Report of a case and analysis of the literature. Arch Derrnatol 99:710-716, 1969. 110. Davidson CL Jr, Winkelmann RK, Kierland RR: Pityriasis rubra pilaris: A follow-up study of 57 patients. Arch Dermatol 100:175-178, 1969. 111. Rook A, Wilkinson DS, Ebling FJG: Textbook of dermatology, ed. 3. Oxford, 1979, Blackwell Scientific Publications, pp. 1295-1298. 112. Leitner ZA, Ford EB: Vitamin A and pityriasis rubra pilaris. Br J Dermatol 59:407-427, 1947. 113. Kint A, de Bie S, Geerts ML, et al: Pityriasis rubra pilaris, a familial condition. Arch Belg Dermatol 158:371-376, 1972. 114. Tarral C: Communication, in Rayer P, editor: A theoretical and practical treatise on the diseases of the skin, ed. 2. London, 1835, Bailli~re, Tindall & Cox, Ltd., p. 648, 115. Devergie A: Trait6 pratique des maladies de la peau, ed. 2. Paris, 1857, Librairie de Victor Masson, pp. 454-464. 116. BesnierE: Observations pour servisal'histoireclinique du pityriasis mbra pilaire. Ann Dermatol Syph 2 (series 10):253-287, 398-427,485-544, 1889. 117. Baden HP, Roth SI: Oral lesions in pityriasis rubra pilaris. Oral Surg 25:691-694, 1968. 118. Lever WF, Schaumburg-Lever G: Histopathology of the skin, ed. 6. Philadelphia, 1983, J. B. Lippincott Co., p. 159, 119. Demis DJ, Dobson RL, McGuire J: Clinical dermatology. Hagerstown, 1979, Harper & Row, Publishers Inc., vol 1, unit 1-13, pp. 1-8. 120. Domonkos AN, Arnold HL, Odom RB: Andrew's Diseases of the skin. Philadelphia, 1982, W. B. Saunders Co., pp. 255-259. 121. Porter D, Shuster S: Epidermal renewal and amino acids in psoriasis and pityriasis mbra pilaris. Arch Dermatol 98:339-343, 1968. 122. Rails IG, Dawber RPR, Ryan TJ, et al: Pityriasis rubra pilaris: Epidermal cell kinetics. Br J Dermatol 104:249252, 1981. 123. Irgang S: Pityriasis rubra pilaris. Responsive to ascorbic acid. Australas J Dermatol 9:211-217, 1968. 124. Anthony TD: Brief clinical reports on two cases of pityriasis rubra pilaris. Australas J Dermatol 5:185-186, 1960. 125. Watt TL, Jillson OF: Pityriasis rubra pilaris: Penicillin and antituberculous drugs as possible therapeutic agents. Arch Dermatol 92:428-430, 1965.

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126. Ashurst PJC: The treatment of pityriasis rubra pflaris with Dipasic. Br J Dermatol 74:372-375, 1962. 127. Birmiek SA: Pityriasis rubra pilaris responding to aminonicotinamide. Arch Derrnatol 114:1348-1349, 1978, 128. Griffiths A, Ralfs I: Aminonicotinamide in pityriasis mbra pilaris. Arch Dermatol 117:127, 1981. (Letter to Editor.) 129. Lamar LM, Gaethe G: Pityriasis rubra pilaris. Arch Dermatol 89:515-522, 1964. 130. Gunther S: Topical administration of vitamin A acid (retinoic acid) in palmar keratoses: Callosities, hyperkeratotic eczema, hypertrophic lichen planus, pityriasis rubra pilaris. Dermatologica 145:344-347, 1972. 131. Ayres S Jr, Mihan R: Pityriasis rubra pilaris controlled by synergism of vitamins A and E. J AM ACAD DERMATOL5:350-351, 1981. (Letter to Editor.) 132. Randle HW, Diaz-Perez JL, Winkelmann RK: Toxic doses of vitamin A for pityriasis rubra pilaris. Arch Dermatol 116:888-892, 1980. 133. Butkus D: Treatment of Darier's disease, lamellar ichthyosis, pityriasis rubra pilaris, cystic acne, and basal cell carcinoma with oral 13-cis-retinoic acid. Dermatologica 157(suppl 1):11-12, 1978. 134. Goldsmith LA, Weinrich AE, Shupack J: Pityriasis rubra pilaris response to 13.cis-retinoic acid (isotretinoin). J A~ ACAD DERMATOL6:710-715, 1982. 135. Lauharanta J, Lassus A: Treatment of pityriasis rubra pilaris with an oral aromatic retinoid (Ro 10-9359), Acta Derm Venereol (Stockh) 60:461-462, 1980. 136, Fleissner J, Happle R: Etretinate in the treatment of juvenile pityriasis mbra pilaris. Arch Dermatol 117:749-750, 1981. 137. Marks R, Finlay AY, Holt PJA: Severe disorders of keratinization: Effects of treatment with Tigason (etretinate). Br J Dermatol 104:667-673, 1981. 138. Beamer JE, Newman SB, Reed WB, et al; Pityriasis rubra pilaris. Cuffs 10:419-423, 1972. 139. Finzi AF, Altomare G, Bergamaschini L, Tucci A: Pityriasis rubra pilaris and retinol-binding protein. Br J Dermatol 104:253-256, 1981. 140. Bergamaschini L, Tucci A, Columbo A, et al: Effect of stanozolol in patients with pityriasis rubra pilaris and retinol-binding protein deficiency. N Engl J Med 306:546-547, 1982. (Letter to Editor.)

Seborrheic dermatitis 141. Domonkos AN, Arnold HL, Odom RB: Andrew's Diseases of the skin. Philadelphia, 1982, W. B. Saunders Co., pp. 218-246. 142. Fitzpatrick TB, Eisen AZ, Wolff K, et ah Dermatology in general medicine, ed. 2. New York, 1979, McGrawHill Book Co., pp. 803-808. 143. Rook A, Wilkinson DS, Ebling FJG: Textbook of dermatology, ed. 3. Oxford, 1979, Blackwell Scientific Publications, pp. 308-312. 144. Demis DJ, Dobson RL, McGuire J: Clinical dermatology. Hagerstown, 1979, Harper & Row, Publishers Inc., vol 1, unit 1-7, pp. 1-10. 145. Barba A, Piubello W, Vantini I, et al: Skin lesions in chronic alcoholic pancreatitis. Dermatologica 164:322326, 1982. 146. Kanwar AJ, Majid A, Garg MP, et al: Seborrheic der-

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147.

148.

149,

150, 151. 152. 153. 154. 155.

matitis-like eruption caused by cimetidine, Arch Dermatol 117:65-66, 1981. (Letter to Editor,) McGinley KJ, Leyden JJ, Marples RR, et al: Quantitative microbiology of the scalp in non-dandruff, dandruff, and seborrheic dermatitis. J Invest Dermatol 64:401-405, 1975. H6ffler U, Gloor M, Peters G, et al: Qualitative and quantitative investigations on the resident bacterial skin flora in healthy persons and in the non-affected skin of patients with seborrheic eczema. Arch Dermatol Res 268:297-312, 1980. Gloor M, Wiegand I, Friederieh HC: Uber Menge und Zusammensetzung tier Hautoberfliiehenlipide beim sogennanten seborrhoischen Ekzem. Dermatol Monatsschr 158:759-764, 1972, Hurwitz S: Clinical pediatric dermatology. Philadelphia, 1981, W, B, Saunders Co,, pp, 14-15, 53-55. Demis DJ, Dobson RL, McGuire J: Clinical dermatology. Hagerstown, 1979, Harper & Row, Publishers Inc., vol 1, unit 1-17, pp. 1-3. Jacobs JC, Miller ME: Fatal familial Leiner's disease: A deficiency of the opsonic activity of serum complement, Pediatrics 49:225-232, 1972. Miller ME, Koblenzer PJ: Leiner's disease and deftciency of C~. J Pediatr 80:879-880, 1972. Mausolf FA: The eye and systemic disease, ed. 2, St. Louis, 1980, The C, V, Mosby Co., pp, 355-357, Thomsen K: Seborrheie dermatitis and napkin dermatitis, Acta Denn Venereol [Suppl] (Stockh) 95:40-42,

Papulosquamous diseases

168. 169. 170.

171. 172. 173, 174. 175.

176.

1981, 156. Ferr~indiz C, Henkes J, Peyri J, et al: Acquired zlne deficiency syndrome during total parenteral alimentation: Clinical and histopathological findings. Dermatologica 163:255-266, 1981. 157. Ackerman AB: Histologic diagnosis of inflammatory skin diseases. Philadelphia, 1978, Lea & Febiger, pp. 239-240, 262, 278-279. 158. Erlichman M, Goldstein R, Levi E, et al: Infantile flexural seborrheic dermatitis. Neither biotin nor essential fatty acid deficiency. Arch Dis Child 56:560-562, 1981. 159. Richardson DP: Seborrheic dermatitis and rosacea. Compr Ther 7:57-60, 1981. 160. Ellis CN, Stawiski MA: The treatment of perioral dermatitis, acne rosacea, and seborrheic dermatitis. Meal Clin North Am 66:819-830, 1982. 161. SolanoE: General dermatologicuseofhydroxamicaeid. Clin Ther 3:229-233, 1980. 162. Parish L: L-dopa for seborrheie dermatitis. N Engl J Meal 283:879, 1970. 163. Burton JL, Cartlidge M, Shuster S: Effect of L-dopa on the seborrhea of Parkinsonism. Br J Dermatol 88:475479, 1973. 164. Kohn SR, Poehi PE, Strauss JS, et al: Sebaceous gland secretion in Parkinson's disease during L-dopa treatment. J Invest Dermatol 60:134-136, 1973, 165. Storrs LA: Management of the ear canal seborrhea with cerumen. Laryngoscope 91:1231-1233, 1981.

Gianotti-Crosti syndrome 166. Hurwitz S: Clinical pediatric dermatology. Philadelphia, 1981, W. B. Saunders Co., pp. 101-103. 167. Claudy AL, Ortonne JP, Trepo C, et al: Acrodermatite

177.

178. 179, 180. 181.

182. 183. 184. 185. 186. 187.

623

papuleuse de l'adulte, Ann DermatoI Venereol 104:190194, 1977. Demis DJ, Dobson RL0 McGuire J: Clinical dermatology, Hagerstown, 1979, Harper & Row, Publishers Ine., vol 2, unit 7-21, pp. 1-2. Gianotti F: Papular aerodermatitis of childhood and other papuloveslcular acro-located syndromes. Br J Dermatol 100:49-59, 1979. Gianotti F: Rilievi di una particolare casistica tossinfettiva caratterizzata da una eruzione eritemato-inflltrativa desquamativa a focolai lenticolarJ, a sede elettiva acroesposta. G Ital Dermatol 96:678-697, 1955. Crosti A, Gianotti F: Dermatose 6ruptive acrositu6e d' origine probablement virosique. Dermatologica 115: 671-677, 1957. Crosti A, Gianotti F: Ulteriore contributo alla conoscenza dell-acrodennatite papulosa infantile, G Ital Dermatol 105:477-504, 1964. DeGaspari G, Bardare M, Constantino D: An antigen in Crosti-Gianotti acrode1~natitis, Lancet 1:1116-1117, 1970. Colombo M, Gerber MA, Vernaee SJ, et al: Immune response to hepatitis B virus in children with papular acrodermatitis, Gastroenterology 73:1103-1106, 1977. Hermans J, Amiel B, Fourcaud G: Aerodermatite papuleuse infantile de Gianotti-Crosti avec Ag Au positif. Evolution vers une h6patite ehronique active, Presse Med 4:2807, 1975. Ishimaru Y, Ishimaru H, Toda G, et ah An epidemic of infantile papular acrodermatitis (Gianotti's disease) in Japan associated with hepatitis B surface antigen subtype ayw, Lancet 1:707-709, 1976. Schneider JA, Poley RJ, Orcutt MA, et al: Papular acrodermatitis (Gianotti-Crostl syndrome) in a child with anicteric hepatitis B, virus subtype adw. J Pediatr 101:219-222, 1982. Lever WF, Sehaumburg-Lever G: Histopathology of the skin, ed. 6. Philadelphia, 1983, J. B. Lippincott Co., pp. 150-151. CasteUano A, Schweitzer R, Tong MJ, et ali Papular acrodermatitis of childhood and hepatitis B infection. Arch Dermatol 114:1530-1532, 1978. Gianotti F: HBsAg and papular acrodermatitis of childhood. N Engl J Med 298:460, 1978. Konno M, Kikuta H, Ishikawa N, et al: A possible association between hepatitis B antigen-negative infantile papular acrodermatitis and Epstein-Burr virus infection. J Pediatr 101:222-224, 1982. JamesWD, OdomRB, Hatch MH: Gianotti-Crosti-like eruption associated with coxsackievirus A-16 infection. J AM AC_ADDERMATOL6:862-866, 1982. Rubenstein D, Estedy NB, Fretzin D: The GianottiCrosti syndrome. Pediatrics 61:433-437, 1978. SpearKL, WinkelmannRK:Gianotti-Crostisyndrome. A review of ten cases not associated with hepatitis B. Arch Dermatol 120:891-896, 1984. Joaquin VH, Marks MI: Gianotti disease or GianottiCrosti syndrome? J Pediatr 101:216-217, 1982. Hjorth N, Kopp H, Osmundsen PE: Gianotti-Crosti syndrome-papular eruption of infancy. Trans St. Johns Hosp Dermatol Soc 53:46-56, 1967. Toda G, Ishimaru Y, Mayumi M, Oda T: Infantile papular aerodermatitis (Gianotti's disease) and intrafamilial

Journal of the American Academy of Dermatology

Fox and Odom

occurrence of acute hepatitis B with jaundice: Age dependency of clinical manifestationsof hepatitis B virus infection. J Infect Dis 138:211-216, 1978.

Lichen aureus 188. Martin RH: Case for diagnosis. Trans St. Johns Hosp Dermatol Soc 40:98, 1958. 189. Calnan CD: Lichen aureus. Br J Dermatol 72:373-374, 1960. 190. Farrington J: Lichen aureus. Cutis 6:1251-1253, 1970. 191. Reinhardt L, Wilkin JK, Tausend R: Vascular abnormalities in lichen aureus. J AMACADDERMATOL8:417420, 1983: 192. Rudolph RI: Lichen aureus. J AM ACADDERMATOL 8:722-724, 1983. 193. Waisman M, Waisman M: Lichen aureus. Arch Dermatol 112:696-697, 1976.

194. Abramovits W, Landau JW, Lowe NJ: A report on two patients with lichen aureus, Arch Dermatol 116:11831184, 1980. 195. Rendall JRS, Robinson T: Lichen aureus. Br J Dermatol 95(suppl 14):45-47, 1976. 196. Ackerman AB: Histologic diagnosis of inflammatory skin diseases. Philadelphia, 1978, Lea & Febiger, p. 211.

Erythrokeratodermia progressiva symmetrica 197. Ruiz-Maldonado R, Tamayo L, del Castillo V, et al', Erythrokeratodermia progressiva symmetrica: Report of 10 eases, Dermatologica 164:133-141, 1982, 198. Gottron HA: Congenital angelegte symmetrische progressive erythrokeratodermie. Z Haut-Geschlechtskr 4:493-494, 1922.

Answers for CME examination*

Identification No. 885-103

March, 1985, issue of the JOURNAL OF THE AMERICAN ACADEMY OF DERMATOLOGY

Questions 1-33, Wagner RF Jr, Tomich JM, Grande DJ: J AM ACAO DERMATOL 12:441-449, 1985. 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11, 12. 13. 14. 15. 16. 17.

e c d a b e e a e b d c c c b a a

(p 441, (p 443, (p 443, (p 443, (p 443, (p 444, (p 444, (p 446, (p 444, (p446, (p447, (p441, (p 441, (p441, (p 444, (p 446, (p 444,

c 1, pa 1) c 2, pa 1; p 445, c 1, pa 1; c 2, pa 1) C 1, pa 2) c i, pa 3) c 2, pa 2) c 1, pa 4) c 1, pa4; c 2, pa 2) c 2, pa 1) c 1, pa 3) c l , pa2) cl, pal) c2, p a l ; p 4 4 2 , TaI) c 2, pa 1; p 442, Ta I) c2, p a l ; p 4 4 2 , TaI) c 2, pa 5) c 2, pa 4) c 1, pa 2)

*p; page; c: column; pa: paragraph; Ta: table,

624

18. 19. 20. 21. 22. 23. 24. 25. 26. 27. 28. 29. 30. 31. 32. 33.

b b a b d a b c c c a d d d a,b,c,e None of the answers is correct

(p 444, (p 444, (p 444, (p 444, (p 444, (p 444, (p 444, (p 447, (p 448, (p 448, (p 448, (p448, (p 448, (p448, (p 447, (p 443,

c 1, e 1, c 1, c 1, c I, c 1, c 2, c 1, c 1, c 1, c 1, cl, c 1, c l, c 2, c 2,

pa 2) pa 2) pa 2) pa 2) pa 2; c 2, pa 5) pa 2) pa 5) pa 3) pa 2) pa 4 - - c 2, pa 1) pa 4 - - c 2, pa 1) pa4 c2, pal) pa 4 c 2, pa 1) pa4 c 2 , p a l ) pa 1--pa 3) pa 3)