Clouston’s Syndrome-A Case Report

Abstract

A heterogeneous group of disorders namely Clouston’s syndromes characterized by a primary defect in teeth, hair, nail and sweat glands with an estimated frequency of about seven per 10,000 births. Hidrotic ectodermal dysplasia or Clouston syndrome is a rare inherited disorder of ectodermal dysplasia. It is an autosomal-dominant disease and is identified by a triad of palmoplantar keratoderma, alopecia and nail dystrophy. At present, there is no treatment for the disease and management is purely supportive. The improved prognosis over time is likely due to greater recognition of the condition. In this report, a 3-year-old child with nail abnormalities and painful thickening & fissuring of palmoplantar skin is reported.