Disseminated superficial actinic porokeratosis: A clinical study

I

I

Disseminated superficial actinic porokeratosis: A clinical study Stephen P. Shumack, MB,BS, and Chris A. Commens, MB,BS, FACD

Westmead, New South Wales, Australia Disseminated superficial actinic porokeratosis is an autosomal dominant condition that requires sun exposure for full expression. It affects only sun-exposed areas, with relative sparing of the face. In sun-damaged skin of Australians disseminated superficial actinic perokeratosis is commonly mistaken for solar keratosis. Twenty-nine subjects with disseminated superficial actinic porokeratosis were involved in an extensive questionnaire and clinical study. The distribution of lesions was charted on all subjects, with a mean count of 268 lesions per subject. We found no evidence that skin cancer had arisen in disseminated superficial actinic porokeratosis lesions. A review of the etiologic and clinical features of disseminated superficial actinic porokeratosis also is presented. (J AM ACAD DERMATOL1989;20:1015-22.) Porokeratosis was first described by MibellP and Respighi 2 in 1893. It is now recognized that there are at least four clinical variants, 3 all of which demonstrate a raised, sharply marginated, keratotie border. The most common form is disseminated superficial actinic porokeratosis, first described as a clinical entity by Chernosky in 1966. 4 It is characterized by multiple small lesions that appear on sun-exposed areas of the body during the third or fourth decade of life. It rarely occurs on the face. Disseminated superficial actinic porokeratosis is inherited as an autosomal dominant condition with reduced penetrance at younger ages. 5 Personal communications with many Australian dermatologists confirm that disseminated superficial actinic porokeratosis is not a rare condition in Australia, although published reports are few.6 In our climate we believe that most subjects with the inherited tendency for disseminated superficial actinic porokeratosis will have sufficient accumulative doses of actinic radiation eventually to express their disease. In contrast, lifelong cumulative doses of actinic radiation may not reach this threshold in other parts of the world. From the Skin and Cancer Foundation. Accepted for publication June 27, 1988. Reprint requests: S. Shumack, MB, BS, The Skin and Cancer Foundation, Suite 17, The Ashley Centre, la Ashley Lane, Westhead N.S.W. 2145, Australia.

SUBJECTS AND METHODS

Twenty-nine subjects (15 women and 14 men) with disseminated superficial actinic porokeratosis were recruited from either the private practice of one of us (C. A. C.) or from their families and studied from April to October 1986. Each subject provided a detailed history, and a total body examination was performed. The history focused on time of onset, progression,extent, and associated features of the lesions of disseminated superficial actinic porokeratosis. The subjects' skin type was evaluated with regard to their tanning response to sunlight.7 Thirteen (45%) had skin type II and 16 (55%) had skin type III. For each subject a sun exposure history was evaluated according to the following guide: (1) life spent actively avoiding the sun--2 subjects (7%); (2) indoor worker with occasional weekend sun exposure---ll subjects (38%); (3) outdoor worker with little leisure sun exposure or indoor worker with marked leisure sun expesure---13 subjects (45%); and (4) outdoor worker with marked leisure sun exposure, "sun worshiper"--3 subjects (10%). All 29 subjects were of a European background with 21 (72%) of Irish, Scottish, or other Celtic ancestry. Physical examination included a count of the number of disseminated superficial actinic porokeratosis lesions at each of the following sites: face, shoulders, back, chest, abdomen, arms, forearms, thighs, legs, and dorsum of hands and feet. The presence of solar damage, as manifest by clinical solar elastosis, actinic keratoses,and basal and squamous cell carcinomas, as well as idiopathic guttate hypomelanosis, was noted. 1015

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Journal of the American Academy of Dermatology

Shumack and Commens

270

It

i

260 250 24(3 230 220 210 200 190 180 170 u~ 160 O 150 u.{ I.L 140 O m 13(3 [L/

01 9

~ 120

9

">'110 10(3 90

80 7(3 60 50

,

i

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oo,o

9r

9

9

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a . .9.

og

4(3 30

20 10 0

I

Legs

~-

[-

Thighs Forearms & Hands

I

I

Upper Shourdars Chest Abdomen Arms & Back

I

Face

Fig. 1. Graph (zero lesions not plotted) of number of disseminated superficial actinic porokeratosis lesions at each specific site on all subjects.

Results were analyzed statistically. Clinical photographs and shave biopsy specimen for histopathologic examination were obtained to confirm the diagnosis. All accessible family members of each subject were asked to undergo examination. RESULTS

The number of lesions on each of the subjects for each evaluated site is shown in Fig. l. The mean number of lesions of disseminated superficial actinic porokeratosis per patient was 268, with a range of 15 to 750. In Table I the mean and standard deviations at each body site are described. The mean age of first recognition of dissemi-

nated superficial actinic porokeratosis was 43 years, with a range of 15 to 78 years. Nineteen (66%) of the subjects had clinical evidence of solar damage as defined previously. Seventeen of 29 subjects (59%) reported or were found to have affected relatives with disseminated superficial actinic porokeratosis; 14 of these had affected members of either sex in a different generation. The differences in the mean values of the subjects (and their significance) related to their sex is described in Table II. Twenty-eight of the subjects (97%) had idiopathic guttate hypomelanosis on their legs or arms (or both). This result is not significantly different

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Disseminated superficial actinic porokeratosis 1017

Table I. N u m b e r of lesions at each specific site Site

Legs Thighs Forearms Upper aspect of arms Back Chest Abdomen Face Whole body

I

Mean

Minimum

Maximum

81 20 79 38 42 6 3 0.2 268

5 0 8 0 0 0 0 0 15

260 66 220 160 250 60 50 5 750

]

SD

66 21 63 43 68 14 12 0.94 209

SD, Standard deviation.

Table II. Variables related to sex of subject Mean values Variable

Men

Age at onset (yr) Sun exposure history Solar damage Skin type Total number of lesions Site of lesions Legs Thighs Forearms Upper aspects of arms Back Chest Abdomen Face

44.0 2.929 0.786 2.6 343 81 22 93 56 72 12 6 0

from that for a group of 50 age-matched control subjects in which 49 had idiopathic guttate hypomelanosis (Shumack SP, Commens CA, unpublished observation). Seventeen (59%) subjects noted that their lesions were more prominent and usually became erythematous and scaly during the summer months. One subject noted a distinct increase in the erythema and prominence of her lesions during pregnancy. Six subjects (21%) stated that heat (without sunlight) made the lesions more prominent. Slight itch was the only symptom, and it occurred in 11 of 29 subjects (38%). Itchiness was noticed mainly during summer or after heat. DISCUSSION Disseminated superficial actinic porokeratosis was first described by Chernosk-y4 i n 1966. He described 12 patients affected by a unique eruption occurring on sun-exposed areas of skin and begin-

I

Women

p value

42.0 2.267 0.533 2.5 198

0.766 0.019 0.164 0.358 0.060

80 18 67 22 14 0.7 0 0.4

0.959 0.571 0.270 0.027 0.018 0.027 0.141 0.260

ning in the third or fourth decade. Individual lesions were small, anhidrotic, and keratotic and had a distinctive rim. Other porokeratotic conditions include: 1. The classic porokeratosis of Mibelli, which comprises a single or a few discrete lesions on the skin or mucous membranes, usually appearing in infancy or childhood. Sometimes lesions may be widely disseminated and less prominent. ~ 2. Porokeratosis palmaris et plantaris disseminata, which usually appears late in the second or third decade of life. It is characterized by many lesions on the palms or soles and the subsequent developmentof lesions similar to disseminated superficial actinic porokeratosis on other areas of the body, including those areas not exposed to sunlight. Porokeratosis punetata palmaris et plataris is probably a forme fruste of this condition,9 with lesions confined to palms and soles. 3. Linear porokeratosis, which usually appears at birth or during childhood. The lesions are arranged in a

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Shumack and Commens

i

Fig. 2. Involvement of legs with disseminated superficial actinic porokeratosis. Arrows mark some larger lesions. linear or zosteriform pattern. It can arise in families affected by disseminated superficial actinic porokeratosis. ~12 Other proposed variants include malignant disseminated porokeratosis, porokeratosis plantaris disereta, and reticulated porokeratosis. The single report of malignant disseminated porokeratosis ~3 probably represents a more widespread and less prominent form of classic plaque porokeratosis in which multiple basal and squamous cell carcinomas had arisen. Porokeratosis plantaris discreta 3 is probably a plantar corn with a keratotic reaction that resembles a cornoid lamella. It is not inherited and appears in adults on pressure points on the soles. Apart from the name, it bears little resemblance to the other forms of porokeratosis. Reticulated porokeratosis 14describes salmon-pink plaques with a reticulated surface and an elevated border. Histologically, there are multiple cornoid lamellae, and apart from this there are no other features to suggest that this condition is a separate porokeratotie variant. It m a y well be that all the true porokeratotic variants are different manifestations of a single, dominantly inherited condition or at

Journal of the American Academy of Derma tology least manifestations of closely linked genetic loci. l0 The mean age of recognition of the disseminated superficial actinic porokeratosis lesions among our subjects was 43 years (range 15 to 78 years) compared with 41.5 years in Anderson and Chernosky's study? It was significant that 8 of our 29 subjects (28%) were unaware of their condition until the time of our examination. Disseminated superficial actinic porokeratosis is usually asymptomatic. It is often difficult to see, particularly in subjects with evidence of sun damage (pigmentary changes, actinic keratoses). Australians often have obvious actinic changes in their skin by the third or fourth decade, so extra lesions (even hundreds) often go unnoticed. As an example, one of our subjects, a 23-year-old woman, had noted her linear porokeratosis lesions for several years but had not noticed lesions typical of disseminated superficial actinic porokeratosis elsewhere on the exposed areas of her body. The genetic pattern of inheritance of disseminated superficial actinic porokeratosis is believed to be autosomal dominant, 5 and it would seem that the sex ratio in our study (15 women and 14 men) is compatible with this. I t should be noted that previous reports of disseminated superficial actinic porokeratosis occurring especially in women 4 may only reflect that women sought advice about the cosmetic disability more frequently than did men. The distribution of lesions of disseminated superficial actinic porokeratosis was confined exclusively to areas exposed to the sun. Lesions were most numerous on the legs, with a mean count of 81 (Fig. 2). The second most common site was the forearms, with a mean count of 79. There was no statistically significant difference between sexes for these two sites (p = 0.96 and p = 0.27, respectively). In Chernosky and Freeman's population, ~5 women, compared to men, had more lesions on the legs, although this was not evaluated statistically. He attributed clothing styles as a possible explanation for this. This does not apply in Australia because men often wear shorts. Compared with women, men have significantly more lesions of disseminated superficial actinic porokeratosis on the upper aspect of the arms, the back, and the chest (p < 0.05). Clothing styles and work patterns explain these differences. Men in Australia often do not wear a shirt outdoors in the summer and are engaged more often than women

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Disseminated superficial actinic porokeratosis

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Fig. 3. Close-up view of lesion of disseminated superficial actinic porokeratosis demonstrating hyperkeratosis around follicularorifice (upper left). On histologicexamination this follicular hyperkeratosis would be seen as an additional cornoid lamella.

in outdoor work or leisure activities. Overall there was a trend for more lesions in men, but this was not statistically significant (p = 0.06). Analysis of lesion numbers at particular body sites could not be correlated with lesion numbers at other body sites. Evidence of solar damage (i.e., elastosis, pigmentary changes, hyperkeratoses, squamous and basal cell carcinomas) and skin type did not show any significant difference between sexes. The face seems to be relatively spared. In fact, only 2 of our 29 subjects had disseminated superficial actinic porokeratosis on the face (patient No. 23 had one lesion; patient No. 26 had five lesions). Similarly, in Chernosky and Freeman's study '5 5 of 31 subjects had facial lesions. We cannot explain the almost complete lack of facial involvement in an area of high sun exposure. A single report ~6 questions the role of actinic damage as a cause of disseminated superficial actinic porokeratosis after the apparent disappearance of some lesions after PUVA therapy. This observation, although perhaps offering a partial explanation for the facial sparing, is not supported by results in our study, which demonstrate total sparing of nonexposed body areas. Other reports have implicated PUVA therapy~7.~8 and light therapy ~9 in the induction of this disease. Sunlight and heat increased the prominence of disseminated superficial actinic porokeratosis

lesions in 17 (59%) of our subjects. Individual lesions uiually became more erythematous, and several subjects reported that the lesions also became more keratotic. This increase in erythema and "scale" often lasted for some weeks after an episode of sun exposure and tended to fade in the winter. In contrast, heat (from any cause) caused only erythema and slight itching lasting less than 1 hour. It seems that the increase in the keratotic material and the prolonged erythema requires sunlight rather than heat alone. This confirms Chernosky's impressions, 4 which suggest that seasonal influences are important. It was difficult to obtain reliable histories about the development of new disseminated superficial actinic porokeratosis lesions. Many subjects had the impression that new lesions arose during the summer. Similarly, many subjects recognized that the lesions became more prominent at that time. The early lesions of disseminated superficial actinic porokeratosis seem to be small, cone-shaped papules, 1 to 3 mm in diameter, usually follicular in location, and topped with a small keratotic plug? The papule then enlarges centrifugally, usually leaving an atrophic central area and a slightly raised, fine keratotic border or rim. This border is usually sharply defined and extends around the entire lesion. The border may be circular but is more often ovoid, petaloid, or irregularly shaped. The centers of the lesions may be hypopigmented,

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Shumack and Cornmens

Fig. 4. Parakeratotic column of cornoid lamella is well visualized. Note also absent granular layer and lymphocytic infiltrate in dermis below lamella. (Hematoxylineosin stain; •

atrophic, hypertrophic, erythematous, or keratotic. Two subjects had several lesions on their backs demonstrating numerous whitish "spines," approximately 1 to 2 mm in height, which appeared to arise from hair follicles in the central area of the disseminated superficial actinic porokeratosis lesion (Fig. 3). This gave an unusual and distinctive appearance. Histologic examination demonstrated multiple cornoid lamellae, concentrated particularly in the follicular orifices. The surrounding rim of the disseminated superficial actinic porokeratosis lesions may sometimes be difficult to visualize because it may be only a fraction of a millimeter in height. A hand lens often proves useful in distinguishing this rim. The lesion is usually 0.5 to 1 cm in diameter, although some of the lesions on some subjects were up to 2 cm. The cornoid lamella is essential for the histologic diagnosis of disseminated superficial actinic porokeratosis and is always present (Fig. 4). It is smaller and lacks the central groove of the cornoid lamella of classic porokeratosis but otherwise

Journal ol the American Academy of Dermatology shares the same histologic features. Reed and Leone ~ postulate that it represents a marker of an abnormal clone of epidermal cells. Wade and Ackerman 2~believe that dermal effects lead to the formation of the cornoid lamella as a manifestation of disordered progression of epidermal cells to cornification. One subject had recurrence of the cornoid lamellae after two shave biopsies, which suggests dermal influences may be important. If a biopsy specimen is obtained, it is important that the sections be cut at right angles to the cornoid lamella. Thus careful marking of the specimen for sectioning is essential. We have demonstrated that in one fourth of disseminated superficial actinic porokeratosis biopsy specimens there are multiple (three or more) cornoid lamellae and that in one third the cornoid lamellae can be seen to be involving appendageal (usually hair follicle) orifices (Shumack S, Commens C, Kossard S, unpublished observation). Evidence supporting the role of actinic damage in the development of disseminated superficial actinic porokeratosis is provided histologically by the frequent occurrence of epidermal atrophy and solar elastosis, both of which are thought to be a result of actinic change. Ultrastructural studies demonstrate changes mainly in the region of the cornoid lamella. These changes include reduplication of the basal lamina at the dermoepidermal junction, vacuolar changes in keratinocytes, dyskeratosis, and parakeratotic cells containing degraded organelles with a reduced granular layer? 2 There have been several reports of more than one type of porokeratosis occurring in the same family l~ or in the same person. ~,~2.~4,25 For expression of porokeratosis we ~~ have postulated that a gene, or closely linked genetic loci, is inherited in an autosomal dominant fashion and a second modulation, either environmental or genetic, is required for the expression of this gene. The various clinical forms of porokeratosis may therefore result from the different types of genetic or environmental modulations that act on the inherited gene. Disseminated superficial actinic porokeratosis could be brought to expression by an accumulated dose of actinic radiation acting on the cells of the subject to cause the expression of this "porokeratotic gene." Malignant change has been reported for classic plaque porokeratosis, 3,13and there have been isolat-

Volume 20 Number 6 June 1989

ed case reports involving linear porokeratosis 26 and disseminated superficial actinic porokeratosis? 7,28 The three cases of squamous ceil carcinoma developing within lesions of disseminated superficial actinic porokeratosis by no means prove a causeand-effect relationship. Two thirds of patients with disseminated superficial actinic porokeratosis in our study have clinical evidence of sun damage in the form of basal or squamous cell carcinoma, solar keratoses, or marked solar elastosis. Disseminated superficial actinic porokeratosis is not a rare condition, and subjects have a large number of lesions (mean 268). Therefore if there were a direct causal relationship, we believe that malignant change would have been observed far more commonly. Treatment of disseminated superficial actinic porokeratosis is unsatisfactory. Ideal therapy should be safe and nonscarring and improve the cosmetic appearance. Cryotherapy with liquid nitrogen is sometimes successful in removing individual lesions but may produce significant hypopigmentation and discomfort. Lesions may also recur after this treatment. Other simple treatments have included topical 5-fluorouracil, which has been reported to be effective 29 in some patients and ineffective in others (Shelley WB, personal communication, 1986). Treatment modalities such as topical retinoids, and retinoids given systemically, 3~ and the CO2 laser 3~ have been reported to be successful in various forms of porokeratosis. The use of these modalities to treat a purely cosmetic disability such as disseminated superficial actinic porokeratosis, especially when a successful result is far from certain, is questionable. Disseminated superficial actinic porokeratosis may be missed at the initial consultation even by the experienced dermatologist. In patients with sun-damaged skin it is commonly mistaken for multiple actinic keratoses. 32 Both conditions are usually bilateral, symmetric, and confined to sunexposed areas. Disseminated superficial actinic porokeratosis, however, rarely involves the face and neck and almost always involves the legs, whereas actinic keratoses have the opposite distribution. 33,34 Disseminated superficial actinic porokeratosis lesions often number in the hundreds, whereas most patients with actinic keratoses have less than 20 lesions. Disseminated superficial actinic porokeratosis also appears at a younger age than do actinic keratoses.

Disseminated superficial actinic porokeratosis 1021 REFERENCES 1. Mibelli V. Contributo allo etudio della ipercheratosi dei canali sudoriferi (porokeratosis). Gior ttal d Mal Ven 1893;28:313-55. 2. Respighi E. Di una ipercheratosi non ancora descritta. Gior Ital d Mal Ven 1893;28:356-86. 3. Chernosky ME. Porokeratosis. Arch Dermatol 1986; 122:869-70. 4. Chernosky ME. Porokeratosis: report of twelve patients with multiple superficial lesions. South Med J 1966; 59:289-94. 5. Anderson DE, Chernosky ME. Disseminated superficial actinic porokeratosis--genetic aspects. Arch Dcrmatol 1969;99:408-12. 6. Donald GF, Hunter GA. Disseminated superficial actinic porokeratosis: a report of eight cases. Australas J Dermatol 1968;9:335-44. 7. Pathak MA, Fitzpatrick TB, Greiter F, et al. Preventative treatment of sunburn dermatoheliosis and skin cancer with sun protective agents. In: Fitzpatrick TB, Eisen AZ, Wolf K, et al., eds. 3rd ed. Dermatology in general medicine. New York: McGraw-Hill, 1987:1509. 8. Marghescu S, Anton-Lamprecht I, Melz-Rothfuss B. Disseminated bilateral hyperkeratotic variant of porokeratosis of Mibelli. Arch Dermatol R.es 1987;279:$38$47. 9. Marshalko M, Somlai B. Porokeratosis plantaris, palmaris, et disseminata. Arch Dermatol 1986;122:890-l. 10. Commens CA, Shumack SP. Linear porokeratosis in two families with disseminated superficial actinic porokeratosis. J Pediatr Dermatol 1987;4:209-14. 11. Welton WA. Linear porokeratosis in a family with disseminated superficial actinic porokeratosis [Letter/. Arch Dermatol 1972;106:263. 12. Dover JS, Phillips TJ, Burns DA, et al. Disseminated superficial actinic porokeratosis: co-existence with other porokeratotic variants. Arch Dermatol 1986;122:887-9. 13. Brodkin RH, Rickert RR, Fuller FW, et al. Malignant disseminated porokeratosis. Arch Dermatol 1987;123: 1521-6. 14. Helfman R J, Poulos EG. Reticulated porokeratosis: a unique variant of porokeratosis. Arch Dermatol 1985; 121:1542-3. 15. Chernosky ME, Freeman RG. Disseminated superficial actinic porokeratosis (D.S.A.P.). Arch Dermatol [967; 96:616-24. 16. Schwarz T, Seiser A, Gschnait F. Disseminated superficial "actinic" porokeratosis. J AM ACADDERMATOL] 984; 11:724-30. 17. Reymond JL, Bean JC, Amblard P. Superficial actinic porokeratosis in a patient undergoing long-term PUVA therapy. Acta Derm Venereol (Stock_h) 1980;60:539-40. 18. Hazen PG, Carney JP, Walker AE, et al. Disseminated superficial actinic porokeratosis: appearance associated with photochemotherapy for psoriasis. J AM Acao DERM~XOL 19P~5;[2:1077-g. 19. Chernosky ME, Anderson DE. Disseminated superficial actinic porokeratosis. Clinical studies and experimental production of lesions. Arch Dermatol 1969;99:401-7. 20. Reed R J, Leone P. Porokeratosis: a mutant clonal keratosis of the epidermis. Arch Dermatol 1979;101: 340-7. 21. Wade TR, Ackerman AB. Cornoid lamellation: a histologic reaction pattern. Am I Dermatopathol 1980;2:515. 22. Jaworski R, Commens C. The ultrastructural appearance

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of disseminated superficiat actinic porokeratosis. Ultrastruct Pathol 1988;12:643-9. Moreland ME, Wyre H W Jr. Porokeratosis: two morphological forms within a family. Arch Dermatol 1981; 117:245-6. Bloom D, Abramowitz EW. Porokeratosis of Mibelli: report of three cases in one family; histologic studies. Arch Dermatol Syph 1943;47:1-15. Dover JS, Miller JA, Levene GM. Linear porokeratosis of Mibelli and D.S.A.P. Clin Exp Dermatol 1986;11:7983. Lozinsld AZ, Fisher BK, Walter JB, et al. Metastatic squamous cell carcinoma in linear porokeratosis. J AM Ac~m DEm~ATOL 1982;16:448-51. Shrum JR, Cooper PH, Greer KE, et al, Squamous cell carcinoma in disseminated superficial actinic porokeratoSiS. J AN ACAD DERMATOL1982;6:58-62. Chemesky ME, Rapini RP. Squamous cell carcinoma in lesions of disseminated superficial actinic porokeratosis: a report of two eases [Letter]. Arch Dermatol 1986; 122:853-4.

29. Shelley WB, Shelley ED. Disseminated superificial porekeratosis: rapid therapeutic response to 5-fluorouracil. Cutis 1983;32:139-40. 30. Pehamberger H, Konrad K. Treatment with an oral aromatic retinoid in linear porokeratosis. Dermatologica 1980;160:270-4. 31. Barnett JI--I. Linear porokeratosis: treatment with the carbon dioxide laser. J AN AcAt~ DERMATOL 1986;14: 902-4. 32. Shumaek SP, Commens CA. Disseminated superficial actinic porokeratosis: a condition often mistaken for actinic keratoses [Letter]. Meal J Aust 1987;147:101-2. 33. Silverstone H, Gordon D. Regional studies in skin cancer. Second report. Wet tropical and subtropical coast to Queensland. Med J Aust 1966;2:733-40. 34. Marks R, Ponsford MW, Selwood TS, et al. Nonmelanotic skin cancer and solar keratoses in Victoria. Med J Aust 1983;2:619-22.

ABSTRACTS

Antibodies against eytomegalovirus and Kaposi's sarcoma Veraldi S, Ferrante P, Barbi M, et al. G Ital Dermatol Venereol 1988;123:75-7 (Italian) To evaluate the role of cytomegalovirus in Kaposi's sarcoma, serum and urine samples of 19 patients with classical Kaposi's sarcoma were taken. IgG and IgM antibodies against cytomegalovirus were evaluated. Viral. isolation studies were also conducted. High titers of IgG antibodies against cytomegalovirus were found in only 1 of 19 cases. Immunoenzymaticexaminations for IgM antibodies against cytomegalovirus yieldednegative findings in all patients. Furthermore, neither eytomegalovirusnor other viruses were isolated from cell cultures. On the basis of the data collected, no relationship was found between cytomegalovirnsand classical Kaposi's sarcoma. Yehudi M. Fe[man, MD

Generalized pustular psoriasis: classification, clinical course and therapy Braun-Falco O, Berthold D, Ruzicka T. Hautarzt 1987;38:50920 (German) On the basis of clinieomorphologic criteria, the authors suggest the classificationof pustular forms of psoriasis into four subtypes: generalized pustular psoriasis(yon Zumbusch) and its atypical forms,erythema annulare centrifugum-like psoriasis with and without pustulation (EACP); psoriasis vulgaris with pustulation; palmoplaatar pustular psoriasis (Konigsbeck-Barber) and its aeral variant; acrodermatitis continua suppurativa (Hallopeau), and transitional forms. This classification takes into consideration both clinical aspects and response to treatment and allows a prognoois of the various types of pustular psoriasis. Because of its mild course and good treatment results, EACP represents a specialform of psoriasis pustulosa generalisata. In the authors' patients, systemicgloeocorticosteroid therapy has proved deletelions, whereasoral photoehemotherapy and etretinate have been found

to be highly effectivemodes of therapy with only mild side effects and have been able to induce even permanent remission in EACP. Yehudi M. Felman, MD

Telangiectatic nodular Kaposi's sarcoma Mossanica N, Brambilla L, Boneschi V. Ital Dermatol Venereol 1987;122:643-6 (Italian) Kaposi's sarcoma is a multifocal malignant neoplasm of endothelial origin that most often affects the skin. Clinical polymorphism is one of its major characteristics. The authors describe a case of Kapooi's sarcoma in which the nodular lesions were surrounded by striking radiating telangiectatic vessels and know of no other such case described in the literature. It is possible that concomitant liver disease in this patient played an important role in the appearance of the telangiectases. Treatment with oral eimetidine and intralesional infiltration of vincristine sulfate caused simultaneous disappearance of the telangiectasesand regression of the nodular lesions. Yehudi M. Felman, MD

Circulating T-lymphocyte subsets in alopecia areata Lutz G, Fritsche C, Bauer R, et al. Aktuel Dermatol 1988; 14:222-36 (German) Th~previously observedreduction in peripheral cytotoxic/suppressor T cell populations in subjects with alopecia areata was confirmed (p < 0.0001). Moreover, a hitherto undescribed disease in circulating natural killer cells was noted (t9 < 0.01). Total IgE was at borderline level in patients without atopy (101.9 kU/L) and, as expected, was substantially increased in those with aIopecia areata associated with atopy (637.0 kU/L). These data establish clearly that disorders of cellular immunity are present in patients affected with alopecia areata regardless of any association of this condition with atopy. Yehudi M. Fehnan, MD