Dermatology Glossary

Hypohidrotic ectodermal dysplasia, X-linked

This X-linked recessive syndrome is characterized by the partial (hypohidrotic) or complete (anhidrotic) absence of sweat glands, hypotrichosis, and hypodontia.
Other facial features include multiple facial deformities and skin wrinkling.
Temporary and permanent teeth are either deformed or absent. Teeth may be conical.
Scalp hair is usually fine and sparse, showing evidence of early breakage.

Treatment:

• Avoidance of excessive heat, in the absence of the ability to sweat, is indicated.
• Genetic counseling is recommended.

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