Clouston�s hidrotic ectodermal dysplasia

Abstract

A 2-year-old girl, from a consanguineous and phenotypically normal family. Her mother who brought her to our institution reporting that the child had hair loss, scanty eyelashes and eyebrows since she was two months old. Physical examination revealed severely dystrophic nails and thin scalp hair, fine eyebrows and eyelashes and thin body hair. Hyperkeratotic and desquamative plaques on her palms and soles. The patient did not present changes in sudoresis, dentition or hearing. So with the above clinical findings, a diagnosis of hidrotic ectodermal dysplasia was made, and the patient treated with skin emollients and topic keratolytics, she is under regular monitoring. Clouston syndrome is a rare genodermatosis that affects skin and annexes, it's belong to ectodermal dysplasias, which occur approximately one in every 100,000 births, which are caused by primary defects in the development of two or more tissues derived from the embryonic ectoderm.