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Wallace H. Clark, Jr, MD

Arch Dermatol. 1988;124(8):1207-1210.

	Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings.

The following constellation of traits constitutes the familial dysplastic nevus syndrome: (1) a distinctive clinical appearance of abnormal melanocytic nevi^1-3; (2) unique histologic features, including an abnormal architectural pattern of intraepidermal melanocytes, cytologically atypical intraepidermal melanocytes, connective tissue changes, and a lymphocytic infiltrate^4,5; (3) an autosomal dominant inheritance, possibly linked to a susceptibility gene on the short arm of chromosome 1 near the Rh locus⁶; and (4) hypermutability of fibroblasts and lymphoblasts. (Fibroblasts and lymphoblasts from patients with the dysplastic nevus syndrome and hereditary cutaneous melanoma are abnormally sensitive to ultraviolet radiation damage, simulated sunlight, and exposure to 4-nitroquinoline-1-oxide.^7,8 Their fibroblasts are also hypersensitive to G₂ chromatid radiation damage.⁹)

The syndrome is, in all likelihood, one of the hypermutability disorders. The familial dysplastic nevus syndrome is presently recognized by its clinical and histologic features, but, in due course, it may . . . [Full Text PDF of this Article]

Author Affiliations
The Pigmented Lesion Study Group Departments of Dermatology and Pathology and the Cancer Center University of Pennsylvania School of Medicine Philadelphia, PA 19104

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