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The scleroatrophic syndrome of Huriez: a cancer‐prone genodermatosis
Author(s) -
HAMM H.,
TRAUPE H.,
BRÖCKER E.B.,
SCHUBERT H.,
KOLDE G.
Publication year - 1996
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1046/j.1365-2133.1996.41773.x
Subject(s) - genodermatosis , medicine , hypoplasia , palmoplantar keratoderma , thenar eminence , dermatology , daughter , keratoderma , pathology , cancer , dyskeratosis , hyperkeratosis , anatomy , biology , biochemistry , evolutionary biology , gene
Summary We report a 24‐year‐old woman, her 6‐year‐old son and her 1 7‐month ‐old daughter, who all suffer from a rare congenital genodermatosis first delineated by Huriez et al. in the 1960s. The clinical features of this autosomal dominant condition include scleroatrophy of the hands and feel, nail hypoplasia, mild palmoplantar keratoderma and hypohidrosis. Histological changes are non‐specific, but immunohistological and ultrastructural examination in our index patient revealed an almost complete absence of epidermal Langerhans cells in the affected skin. This new finding may be linked to the cancer proneness of the scleroatrophic skin. In this family, the grandmother had died at the age of 37 years from metastatic squamous cell carcinoma which had arisen on the thenar eminence.