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Image Number 351 (Scleroatrophic syndrome of Huriez)


Diagnosis: Scleroatrophic syndrome of Huriez

Site: Hand,palm

Sex: M

Age: 16

Type: Clinical

Description: Keratoderma

Submitted by: Shahbaz Janjua

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Differential Diagnosis


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.






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