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November 1987

Epidermodysplasia VerruciformisA Case Associated With Primary Lymphatic Dysplasia, Depressed Cell-Mediated Immunity, and Bowen's Disease Containing Human Papillomavirus 16 DNA

Author Affiliations

From the Department of Microbiology and the Institute of Human Genetics, School of Medicine, University of Minnesota, Minneapolis (Drs Ostrow and Faras and Ms Manias), and the Department of Dermatology, Henry Ford Hospital, Detroit (Drs Mitchell and Stawowy).

Arch Dermatol. 1987;123(11):1511-1516. doi:10.1001/archderm.1987.01660350111025

• Epidermodysplasia verruciformis is a rare, often hereditary disease characterized by a generalized cutaneous infection with human papillomavirus (HPV), depressed cell-mediated immunity, and a propensity for transformation of the warty lesions to squamous cell carcinoma on primarily sun-exposed areas of the skin. A 37-year-old man with congenital lymphatic dysplasia and a history of squamous cell carcinoma of the groin and foot was observed by us to have edema of all four extremities, numerous flat warts, and pityriasis versicolor-like papules over the trunk and arms. Condylomatous lesions were noted in the groin and a periungual verrucous nodule on the thumb. Biopsies showed the trunk and arm lesions to be verrucae and the thumb lesion to be Bowen's disease. Results of molecular hybridization studies from four lesions of the arms showed the presence of only HPV 3 DNA; HPV 16-related DNA was detected in the intraepidermal carcinoma on the thumb. Immunologic evaluation revealed anergy to routine skin testing, depressed mitogen-stimulated lymphocyte transformation, decreased B-lymphocyte count, and a severe reversal of the T-lymphocyte helper:suppressor ratio.

(Arch Dermatol 1987;123:1511-1516)