Sclerotylosis, Huriez Syndrome, Keratoderma, Palmoplantar With Sclerodactyly, Keratoderma, Palmoplantar - DermAtlas

Contributor

Eric Ehrsam, MD

Diagnosis

sclerotylosis Huriez syndrome keratoderma, palmoplantar with sclerodactyly keratoderma, palmoplantar

Body Site

finger

Age

17 years

Pigmentation

medium

Organization

confluent

Comments

A 17-year old man developed a palmo-plantar keratoderma associated with acrosclerosis and nail atrophy of three toes. Many members of his family were affected by this syndrome. Sclerotylosis or Huriez Syndrome is a genodermatosis characterized by sclerodactyly, nail anomalies, and palmar-plantar keratoderma. Other associated findings include telangiectasias of the lip, flexural contractures of the fifth finger, and poikiloderma of the nasal skin. This young man was a member of the family described by Delaporte (Delaporte E et al: Keratoderma with scleroatrophy of the extremities or sclerotylosis (Huriez syndrome): a reappraisal. Brit. J. Derm. 1995; 133: 409-416)

Description

atrophy, sclerosis, and scale

Image Added

10/11/2004 8:09:45

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