A Rare Case of Hypohidrotic Ectodermal Dysplasia in a Seven-Year-Old Child

Abstract

Ectodermal dysplasias (EDs) encompass a large group of inherited disorders that affects two or more ectodermally derived structures. Hair, sweat glands, teeth, and nails are the most common ectodermal derivates affected. Other ectodermal structures that may be affected are ears, eyes, lips, and mucous membranes of the mouth or nose. During embryonic development, the ectoderm forms the outermost layer of the primary germ layers that give rise to the several structures that are commonly affected in ED. Therefore, ED manifests differently among patients, depending on the abnormality's combination and severity. Out of 150 distinctive syndromes, the most common syndromes within this group are hypohidrotic (defective sweat glands) and hidrotic (normal sweat glands). In addition, different types of inheritance patterns are found in ED; X-linked inheritance is by far the most common mode of inheritance. We present here the clinical case of hypohidrotic (anhidrotic) ED in a seven-year-old boy.