Wednesday, March 30, 2005

Palmoplantar keratoderma, sclerodactyly and nail dystrophy

Scleroatrophic syndrome of Huriez is a rare congenital genodermatosis of autosomal dominant inheritance, characterized by scleroatrophy of the hands and feet, nail hypoplasia, palmoplantar keratoderma and hypohidrosis. This 16-year-old boy was born with mild PPK that slowly progressed and worsened alongwith the development of sclerodactyly and nail dystrophy. The development of aggressive squamous cell carcinoma of the affected skin is distinctive feature of the syndrome, occurring in >15% of affected individuals. SCC in Huriez syndrome is characterized by early onset, mostly in the third to fourth decade of life, and by early metastasis. What treatment modalities do you suggest for the severe keratoderma associated with this condition? View images

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