Scleroatrophic syndrome of Huriez in an infant.
We present a 3-year-old boy with scleroatrophic syndrome of Huriez, a rare autosomal dominant condition with only seven affected families worldwide. Although assumed to occur from birth, an evolving case has not previously been documented. Infants do not possess the focal areas of keratoderma on the palms or soles; these develop in adult life. Of particular interest is the high incidence of squamous cell carcinomas that arise from the scleroatrophic skin.
Downs
A M
AM
Department of Dermatology, Bristol Royal Infirmary, England.
Kennedy
C T
CT
D000328:Adult; D002294:Carcinoma, Squamous Cell / Q000235:genetics*; D002675:Child, Preschool; D005500:Follow-Up Studies; D006229:Hand Dermatoses / Q000175:diagnosis / Q000235:genetics*; D006801:Humans; D007645:Keratoderma, Palmoplantar / Q000175:diagnosis / Q000235:genetics*; D008297:Male; D009260:Nail Diseases / Q000175:diagnosis / Q000235:genetics*; D010375:Pedigree; D012594:Scleroderma, Localized / Q000175:diagnosis / Q000235:genetics*; D012878:Skin Neoplasms / Q000235:genetics*; D013577:Syndrome