RT Journal
A1 Clark WH, Jr
T1 THe dysplastic nevus syndrome
JF Archives of Dermatology
JO Archives of Dermatology
YR 1988
FD August 1
VO 124
IS 8
SP 1207
OP 1210
DO 10.1001/archderm.1988.01670080019010
UL http://dx.doi.org/10.1001/archderm.1988.01670080019010
AB The following constellation of traits constitutes the familial dysplastic nevus syndrome: (1) a distinctive clinical appearance of abnormal melanocytic nevi1-3; (2) unique histologic features, including an abnormal architectural pattern of intraepidermal melanocytes, cytologically atypical intraepidermal melanocytes, connective tissue changes, and a lymphocytic infiltrate4,5; (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