Vulvar melanosis and lentiginosis: A case report

Volume 27 Number 5, Part 1 November 1992

Brief communications I

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Vulvar melanosis and lentiginosis: A case report Lina F. Kanj, MD, a Nelly G. Rubeiz, MD, a Adnan M. Mroueh, MD, b and Abdul-Ghani Kibbi, MD Beirut, Lebanon Vulvar melanosis and/or lentiginosis (VM/L) is a cause of concern for patients and clinicians, t3 These lesions may be single or multiple and are characterized by large size, irregular borders, and variegated pigmentary patterns: Despite their clinical appearance, the histologic features are benign. t, 3-8 Little is known about the biologic behavior of V M / L . However, during the past few years 17 cases have been reported; malignant melanoma has not occurred in any of these. We report the case of a woman with multiple pigmented macules on her genitalia. CASE REPORT

A 42-year-old white woman had asymptomatic brown to deep brown patches on her genitalia of 4 years' duration. The lesions were increasing in size and number. The patient was healthy and had no history of drug intake other than oral contraceptives. There was no family history of similar lesions. Examination revealed several discrete and confluent dark brown macules on the labia majora and the introitus. These macules ranged from a few millimeters to several centimeters, with irregular borders and skip areas (Fig. 1). A biopsy specimen demonstrated elongation and anastomosis of the lower portion of the epidermis. Slight melanocytic hyperplasia was observed, but no atypical melanocytes were noted (Fig. 2). In the dermis, pigment-laden macrophages were present without an accompanying dermal inflammatory cell infiltrate. DISCUSSION Our patient conforms to the description of cases that have been reported under the term vulvar melanosis and~or lentiginosis, t'8 To our knowledge, only 18 cases of V M / L , including ours, have been reported. 1, 3, 4, 7 Several benign and malignant conditions must be excluded in the differential diagnosis of these macular hyperpigmented genital lesions. Seborrheic From the Departments of Dermatolog/~ and Obstetrics and Gynecology,b American University of Beirut Medical Center. No reprints available. 16/54/38330

Fig. 1. Close-up of irregular dark brown macules over labia and introitus. keratosis, melanocytic nevi, fixed drug eruption, and postinflammation hyperpigmentation can be distinguished by histologic examination, t,3 Clinically, V M / L may be confused with early patch-stage malignant melanoma, l, 3, 4, 6, 7 However, V M / L lacks the atypical melanocytic hyperplasia characteristic of early patch-stage malignant melanoma. In addition, the pigmentation is mostly exhibited in the basal cell layer in V M / L , whereas it is present at all levels in a haphazard fashion in malignant melanoma. Finally, nesting, invasion of the adnexae, and mitotic figures are absent in V M / L . 3, 7 Pigmented Bowen's disease and patch-type bowenoid papulosis are the other malignant conditions that can mimic V M / L . Both can be distinguished histologically by the presence of parakcratosis, disorderly arrangement of atypical keratinocytes, dyskeratosis, and extension into follicular infundibulae), 9 Penile counterparts of these distinctive macular pigmented lesions of the female genitalia have been reported in 15 men, 4, 5,6,8, lo Melanocytic atypia was present in one patient with no long-term followup) ~ The lesions otherwise seemed to be similar clinically and histologically to V M / L . However, prominent dendritic processes of melanocytes were uniformly present in the l0 cases reviewed by Barnhill et al. 4 but not in the other five cases reported earlier. 8

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Journal o[ the American Academy of Dermatol%y

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Fig. 2. Melanocytic hyperplasia in basal cell layer. (Melanin stain; x400.) It is probable that histopathologicaUy this entity may actually be a spectrum; at one end there is hypermelanosis of the epidermis without melanocytic hyperplasia, whereas at the opposite end there is atypical melanocytic hyperproliferation. 6 V M / L appears to have a benign course with no reports of malignant degeneration. The sparsity of case reports and the lack of long-term follow-up, however, make it impossible to predict its natural history. Biopsies should be performed whenever pigmented spots are noted in the genital area. Regular follow-up and repeat biopsies of changing lesions are suggested until this condition is understood more completely.

2. Maize JC. Mucosal melanosls. Dermatol Clin 1988;6:28393. 3. Rudolph RL Vulvar melanosis. J AM ACAD DERMATOL 1990;23:982-4. 4. Barnhill RL, Albert LS, Sharna SK, et al. Genital lentiginosis: a clinical and histopathologic study. J AM ACAD DERMATOL 1990;22:453-60. 5. Kopf AW, Bart RS. Penile lentigo. J Dermatol Surg Oncol 1982;8:637-9. 6. Leicht S, Youngberg G, Diaz-Miranda C. Atypical pigmented penile maeules. Arch Derrnatol 1988;124:1267-70. 7. Sison-Torre EQ, Ackerman AB. Melanosis of the vulva: a clinical simulator of malignant melanoma. Am J Dermatopatho/1985;7(suppl):51-60. 8. Revuz J, Clerici T. Penile melanosis. J AM ACAD DERMA. TO1. 1989;20:567-70. 9. Lever WF, Schamburg-Lever G. Bowen's disease. In: Histopathology of the skin. 7th ed. Philadelphia: JB Lippincott, 1990:549-51. 10. Bhawan J, Cahn TM. Atypical penile lentigo. J Dermatol Surg Oncol 1984;10:99-[00.

REFERENCES 1, Jackson R. Melanosis of the vulva..I Dermatol Surg Oneol 1984; 10:119-21. I

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PUVA treatment of pigmented purpuric lichenoid dermatitis (Gougerot-Blum) Judit Krizsa, MD, J~mos Hunyadi, PhD, and Attila Dobozy, DSc Szeged, Hungary The term pigmented purpuric dermatosis incorporates several diseases characterized by orange or brown pigmentation interspersed with From the Department of Dermatology, Albert Szent-Gy6rgylMedical University. Reprint request.s: Judit Krizsa, MD, P.O. Box 480, H-6701 Szeged, Hungary. 16/54/38765

petechiae. One of these is the pigmented purpuric lichenoid dermatitis (PPLD) described by Gougerot and Blum. The disease most often occurs on the legs. t Pigmented purpuric dermatoses can be resistant to treatment. Simon and Hunyadi 2 reported the PUVA therapy to be effective in eczematid-like purpura; this was recently confirmed by Wong and Ratnam. 3