A Rare Case of Hypohidrotic Ectodermal Dysplasia

Abstract

A wide range of hereditary diseases affecting two or more ectodermally derived tissues together are referred to as ectodermal dysplasias (EDs). The most frequently impacted ectodermal derivatives are the teeth, nails, sweat glands, and hair. The mucous membranes of the mouth and nose, lips, eyes, ears, and other ectodermal structures could also be impacted. 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. As a result, depending on the array and severity of the anomaly, ED presents itself differently in each patient. This symptom is present in 1 in 50,000 people worldwide. Among these 150 unique syndromes, hypohidrotic (faulty sweat glands) and hidrotic (normal sweat glands) syndromes are the most prevalent. Moreover, there are many inheritance patterns associated with ED, with X-linked inheritance being by far the most prevalent. Here, we report on a seven-year-old boy's clinical case of hypohidrotic (anhidrotic) ED. Keywords: Hypo Hidrotic Ectodermal Dysplasia, Hair, Skin, Oral Cavity, Xerosis, Hypodontia