Ann Dermatol.  1998 Apr;10(2):91-96. 10.5021/ad.1998.10.2.91.

Two Cases of Anhidrotic Ectodermal Dysplasia with Atopic Dermatitis in Siblings

• Kim HS
• Oh CW

• KMID: 2359800
• DOI: http://doi.org/10.5021/ad.1998.10.2.91

Abstract

Anhidrotic ectodermal dysplasia (AED) is characterized by a well-known tetrad of anhidrosis, hypotrichosis, hypodontia, and typical facies with a wide constellation of developmental defects of tissues derived from the ectoderm. Most of these patients have normal or borderline normal intelligence, but some present with mental retardation. A 15-year-old boy and his younger brother were evaluated for dry skin and intolerance to heat since their births. Their parents and other brother were normal. Both of them had atopic dermatitis. A skin biopsy was done on their left axilla, which showed a few immature eccrine glands in the dermis. We report herein two rare cases of AED in siblings with atopic dermatitis.

Keyword

Anhidrotic ectodermal dysplasia

MeSH Terms

Adolescent
Anodontia
Axilla
Biopsy
Dermatitis, Atopic*
Dermis
Eccrine Glands
Ectoderm
Ectodermal Dysplasia*
Facies
Hot Temperature
Humans
Hypohidrosis
Hypotrichosis
Intellectual Disability
Intelligence
Male
Parents
Parturition
Siblings*
Skin