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20. 21. 22.
Herpes simplex infection resembling mechanobullous disease
anomalies and herpes virus infection. Am J Dis Child 126:364-366, 1973. Schaffer AJ, Avery ME: Diseases of the newborn, ed. 3. Philadelphia, 1971, W. B. Saunders Co., p. 656. Whitley RJ, Nahmias AJ, Visintine AM, et al: The natural history of herpes simplex virus infection of mother and newborn. Pediatrics 66:489-494, 1980. Meyers JD, Wade JC, Mitchell CD, et al: Multicenter collaborative trial of intravenous acyclovir for treatment of mucocutaneous herpes simplex virus infection in the immunocompromised host. Am J Med 73(Suppl 1A): 229-235, 1982. Nahmias AJ, Keyserling HL, Kerrick GM: Herpes simplex, in Remington J, Klein J, editors: Infectious diseases of the fetus and newborn infant. Philadelphia, 1983, W. B. Saunders Co., pp. 636-678.
24. Nahmias AJ, Josey WE, Naib ZM, et al: Perinatal risk associated with maternal genital herpes simplex virus infection. Am J Obstet Gynecol 110:825-837, 1971. 25. Moseley RC, Corey L, Benjamin B, et al: Comparison of viral isolation, direct immunofluorescence, and indirect immunoperoxidase techniques for detection of genital herpes simplex virus infection. J Clin Microbiol 13:913-918, 1981. 26, Solomon AR, Rasmussen JE, Varani J, et al: The Tzanek smear in the diagnosis of cutaneous herpes simplex. JAMA 251:633-635, 1984. 27. Mannino F, Robb JA, Benirsehke K: Inapparent intrauterine HSV infection detected by immunohistochemistry. Pediatr Res 19:280, 1984. (Abst.)
I II Ill
Eruptive widespread Spitz nevi Steven A. Smith, M . D . , * Calvin L. Day, Jr., M . D . , and Darl E. Vander Ploeg, M.D. San Antonio, TX A 12-year-old girl was evaluated for a widespread eruption of Spitz nevi. Multiple Spitz nevi are rare, and this variant is exceedingly rare. The reported cases of eruptive nevi of all types are briefly reviewed. Very little is known regarding cause, natural history, or treatment. Cutaneous stimulation occurring in a genetically predisposed individual is the proposed essential causative factor in this disorder. (J AM ACAD DERMATOL15:1155-1159, 1986.)
In 1948 Dr. Sophie Spitz established the histologic criteria for the differentiation of benign juvenile melanoma f r o m malignant melanoma. Since that time, there have been at least seven series of forty patients or more in the literature involving more than 900 total cases. 2"5 All cases have had solitary lesions except for twenty-five cases with multiple lesions. 6~6 O f these cases, all but three have been of the agminated variety (lo-
From the Department of Medicine, Division of Dermatology,University of Texas HealthScienceCenter at San Antonio. Reprint requests to: Dr. StevenA. Smith, Divisionof Dermatology, Oral Roberts University, 8181 South Lewis, PO Box 707070, Tulsa, OK 74170-7070. *PresentlyChiefof the DermatologyDivision,Departmentof Internal Medicine,OralRobertsUniversitySchoolof Medicine,Tulsa, OK.
cally clustered together). 613 We n o w report the fourth case, to our knowledge, ever described o f widespread eruptive Spitz nevi. 14-16 CASE REPORT A 12-year-old girl was seen in 1983 with a 2-year history of numerous widespread asymptomatic pigmented papules and nodules (Fig. 1). These lesions had erupted after a tonsilectomy, postoperative fever, and an erythematous eruption of unknown cause that had resolved. The clinical morphologic picture varied dramatically. There were verrucoid hyperkeratotic papules, heavily pigmented dome-shaped nevoid papules, pink domeshaped papules, and flesh-colored polypoid papules (Fig. 2). The lesions were most heavily concentrated on the buttocks and thighs. However, they were present on all body locations except the palms, soles, and scalp.
Journal of the American Academy of Dermatology
S m i t h et al
', ,'~:,,2 ,'2,rr ,~
Fig. 1. Over seventy-five lesions on the posterior aspect of the thighs (biopsy sites marked). ]Fig. 2. Close-up view showing variable morphologic picture (biopsy sites marked). ]Fig. 3. Acanthosis and pleomorphic nevus cells in the epidermis and papillary dermis, with maturation deeper in the dermis. ]Fig. 4. Cellular and nuclear pleomorphism and atypia at the dermoepidermal junction.
Biopsy of each type of lesion showed the same findings--hyperkeratosis, acanthosis, and large pleomorphic spindle and epithelioid nevus cells in the epidermis and papillary dermis with "maturation" of histologic features in the reticular dermis (Figs. 3 and 4). All four biopsies showed convincing evidence of Spitz nevi. Slowly, an occasional new lesion would appear and an occasional old lesion would flatten and finally disappear. There was no family history of unusual pigmented lesions, and none of the lesions had been previously treated. The patient remains under observation
at present, and no specific treatments have been attempted. COMMENT
The clinical differential diagnosis included several other entities in addition to eruptive Spitz nevi. These included eruptive nevocytic nevi, multiple juvenile xanthogranulomas, urticaria pigmentosa, and dysplastic nevus syndrome, as well as metastatic malignant melanoma. Only eruptive nevi were seriously considered.
Volume 15 Number 5, Part 2
T a b l e I. Four cases of widespread eruptive Spitz nevi Age at onset Author
[ No. of (yr) I lesions
Duration of disease Skin
Appearance and disappearance x 22 yr; slowing of new lesions x 4 yr Slow appearance of new lesions x 6 yr; considerable regression at 13 yr Not stated
Multiple Palms and soles spared
Capetanakis ~6 M
Multiple Thighs, buttocks, arms, legs, trunk
(1975) Burket 14
(1979) Our case
Palms, soles, chest, and scalp spared Multiple Palms, soles, and scalp spared
Diffuse, nonspecific Abnormal EEG
Seizures, bilateral Moderately abnormal EEG
Excellent cosmetic resuits with cryotherapy Slow appearance and Preceding tonsilectomy, disappearance of fever, rash lesions × 2 yr
The details of the four reported cases are compared in Table I. Additionally, a case of multiple lesions on the left thigh and buttock, reported by Bourlond 9 in 1971, has been mistakenly reported as having occurred bilaterally (widespread). ~6 However, after careful translation, this case clearly represents a localized unilateral type (agminated). Understandably, not much is known about this entity; however, four features are of interest. First, the initial onset is eruptive, and then there is a smouldering course over a variable number of years before the process regresses. Second, the initiating factor or factors are purely speculative. Operative and/or postoperative stresses seemed to be associated with our case. Sunburn, radiotherapy, and trauma have been associated with a few cases of the agminated variety (see paragraphs 5 to 7 of this section). 6'8'1~ Third, there is some possibility of extracutaneous lesions in two widespread cases with electroencephalographic abnormalities. Fourth, the treatments are all unsatisfactory in some way--excision is impractical, electrosurgery is temporary, and cryosurgery leaves nevus cells present in the dermis. ~4.~6 To our knowledge the entire subject of eruptive nevi of the various types has not been reviewed. It has relevance here because a common pathophysiologic thread may be present. However, when
Table II, Histologic evidence of Spitz nevi Feature of Spitz nevi
Hyperkeratosis and/or hypergranulosis Acanthosis and/or elongation of rete ridges Large pleomorphic nevus cells superficially "Maturation" of nevus cells at lower levels Increased and/or dilated blood vessels Sparse melanin
I I~bert~ Sanderson ~s Early et al t9 ( 1 9 3 8 ) (1960) 1 (1977) + +
there are so few cases, the thread is indeed stretched very thin. We are able to glean three additional cases reported from 1938 to the present as "multiple pigmented nevi," "eruptive telangiectatic cellular naevi," and "eruptive nevi" that may well represent cases of widespread eruptive Spitz nevi. t7-19 (See Table II for histologic evidence.) One patient had a transient loss of consciousness just preceding onset of the nevi, 18 but no other associated factors were described. One
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o f us (S. A. S.) reviewed the literature from the turn o f the century and found one case of widespread eruptive lentigines following measles and one case o f widespread eruptive cellular nevi following typhoid fever. 18 Coskey2° described three cases of widespread eruptive simple lentigines, junctional nevi, and compound nevi without any associated preceding stimuli. However, from 1977 to 1979, four patients were reported with widespread eruptive nevocytic nevi following erythema multiforme and toxic epidermal necrolysis? la3 Nevi developed in areas of previous bullae. These authors 21-23 discussed possible mechanisms, including melanocytic seeding of injury sites, increased melanocyte-stimulating nerve growth factor, or decreased melanocyte chalone in the locally altered environment. Although these eruptive nevi are clearly not of the Spitz variety, they offer additional data in a field with few data available. Finally, a case of eruptive blue nevi was reported in 1981 )4 The nevi developed from an agminated cluster of brown " f r e c k l e s " that had developed 3 to 4 years previously following a severe, blistering sunburn. A localized response to cutaneous injury, somehow stimulating a developmental residue of dermal melanocytes, was postulated. The common (but not universal) thread does seem to be a preceding cutaneous injury. Both exogenous and endogenous sources of injury have been amply described. Certainly, melanocyte and nevus cell proliferation is seen frequently by dermatologists and is induced frequently by common dermatologic therapies, 21,2~ Little is known, however, concerning specific control mechanisms. We propose that subclinical nests of nevus cells may occur in some individuals on a genetic and/or developmental basis. Thereafter, only when these " p e c u l i a r " people experience the appropriate " p e c u l i a r " cutaneous stimulation would they manifest the eruptive nevi. This manifestation could occur either on a local basis (agminated) or on a systemic basis (widespread) with either a local or a systemic stimulus. To our knowledge, no model currently exists to test this idea. Therefore we have presented some brief observations and hypotheses on a rare but fascinating group of diseases. Only further investigation and long-term follow-up of a considerably
larger number of cases of eruptive nevi will bring reliable understanding of the cause, natural history (including malignancy potential), and treatment. Rydelle Labs, Palo Alto, CA, generously provided for the color reproductions. REFERENCES
1. Spitz S: Melanomas of childhood. Am J Pathol 24:591609, 1948. 2. Allen AC: Juvenile melanomas. Ann NY Aead Sci 100:29-46, 1963. 3. KopfAW,AndadeR: Benignjuvenilemelanoma, inKopf AW~AndradeR, editors:Year book of dermatology.Chicago, 1966, YearBook Medical Publishers, Inc., pp. 752. 4. Weed0n D, Little JH: Spindle and epithelioid cell nevi in children and adults. Cancer 40:217-225, 1977. 5. Paniago-PereiraC, Maize JC, Aekerman AB: Nevus of large spindle and/or epithelioid cells (Spitz nevus). Arch Dermatol 114:1811-1823, 1978. 6. Traub EF, Lewis GM: Melanoma. Arch Dermatol Syphilol 59:349-350, 1946. 7. BrownsteinWE: Multipleagminatedjuvenile melanoma, Arch Dermatol 106:89-91, 1972. 8. Weimar VM, Zuehlke RL: Multiple agminate spindle and epithelioid cell nevi in an adult. Arch Dermatol 224:1383-1384, 1978. 9. Bourlond A: Multiple juvenile Melanome. Hautarzt 22:144-149, 1971. 10. GouldDJ, BleehenSS: Multipleagminatedjuvenile melanoma. Clin Exp Dermatol 5:63-65, ! 980. 11. Krakowski A, "fur E, Brenner S: Multiple agminated juvenile melanoma: A case with a sunburn history, and a review. Dermatologica163:270-275, 1981. 12. Lancer HA, Muhlbauer JE, Sober AJ: Multiple agminated spindle cell nevi: Unique clinical presentation and review, J AM ACADDERMATOL8:707-711, 1983. 13. ProseNS, HeilmanE, FelmanYM, et al: Multiplebenign juvenile melanoma. J AM ACADDERMa'rOE9:236-242, 1983. 14. Burket JM: Multiple benign juvenile melanoma. Arch Dermatol 115:229, 1979. 15. Wallace HJ: Eruptive juvenile melanomata. Br J Dermatol 91(suppl 10):37-38, 1974. 16. Capetanakis J: Juvenile melanoma disseminatum. Br J Dermatol 92:207-211, 1975. 17. EbertMH: Multiplepigmentednevi. Arch Dermatol Syphilol 37:1-21, 1938. 18. Sanderson KV: Eruptive telangiectatic cellular nevi. Br J Dermatol 72:302-307, 1960. 19. EadyRAJ, Gilkes JJH, Jones EW: Eruptive nevi: Report of two cases, with enzymehistochemical,light and electron microscopical findings. Br J Dermatol 97:267-278, 1977. 20. Coskey RJ: Eruptive nevi. Arch Dermatol 111:1658, 1975, 21. Kopf AW, Grupper C, Baer R, Mitchell JC: Eruptive nevocytic nevi after severe bullous disease. Arch Dermatol 113:1080-1084, 1977. 22, GoerzG: EruptivenevocyticneviafterLyell's syndrome. Arch Dermatol 114:1400-1401, 1978.
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23. Soltani K, Bernstein JE, Lorincz AL: Eruptive nevocytic nevi following erythema multiforme. J" AM ACADDERMATOL1:503-505, 1979.
24. Hendricks WM: Eruptive blue nevi. J AM ACAD DERMATOL4:50-53, 1981.
Pustular folliculitis associated with
Demodex folliculorum* Stephen M. Purcell, Captain, MC, USAF, Thomas J. Hayes, Major, M C , U S A F , and Steven L. Dixon, Major, MC, USAF Lackland Air Force Base, TX A 49-year-old man presented with an acute onset of folliculitis on his right cheek. The folliculitis was unresponsive to antibiotics. A potassium hydroxide preparation of a scraping from the affected area revealed the presence of numerous Demodex mites. The patient was treated with topical crotamiton (Eurax) cream resulting in rapid resolution of the folliculitis. We believe that this case represents a localized pustular folliculitis resulting from a heavy infestation with Demodex folliculorum. In spite of more than 50 years of investigation, the role of D. folliculorum in human cutaneous disease remains controversial. (J AM ACAD DERMATOL15:1159-1162, 1986.)
Demodexfolliculorum is the most common ectoparasite of man.' Because of its prevalence on human skin, its role as a pathogen remains a controversial issue. To this date there is no critical scientific evidence to indict this follicular mite as the causative agent of human disease. There have been, however, numerous clinical observations linking the presence o f extraordinary numbers of Demodex mites with various skin disorders. Demodex has been suggested as the etiologic agent in a papulopustular scalp eruption, 2 pityriasis folliculorum, 3 a " d r y " variant of acne rosacea, ~ a perioral granulomatous dermatitis, 4 and blepharitis. 5'6 We have recently seen a patient with a loFrom the Departmentof Dermatology/SGHMD,WilfordHallUSAF Medical Center. Reprint requests to: Dr. Stephen M. Purcell, DermatologyService/ SGHMD, EhrlingBergquistUSAFRegionalHospital, OffuttAir Force Base, NE 68113. *The opinions or assertions contained herein are the private views of the authorsand are not to be construedas officialor as reflecting the views of the Departmentof the Air Force.
calized pustular folliculitis associated with a heavy infestation o f Demodex mites. Localized pustular folliculitis should be added to the list o f dermatoses ascribed to infestation with D. folliculorum. CASE R E P O R T
A 49-year-old Latin man complained of a mildly pruritic eruption present on his right cheek for 48 hours. The patient had been seen in the emergency department on the previous day and treated with oral dicloxacillin for "probable folliculitis." Medications at the time of presentation included hydrochlorothiazide and metoprolol tartrate for hypertension, allopurinol for asymptomatic hyperuricemia, and ibuprofen for osteoarthritis. The patient was also in the eighth day of a course of erythromycin for a mild paronychia that had resolved. The patient denied a previous history of similar eruptions or contact with known allergens or irritants. Examination revealed a 3-cm erythematous, edematous plaque studded with 1-2 mm follicular pustules located on the right cheek (Figs. 1 and 2). There were several scattered 2-3 mm erythematous papules and fol1159