Aberrant scalp hair patterning in hypohidrotic ectodermal dysplasia
Smith, D.W.; Knudson, R.W.
Journal of Pediatrics 90(2): 248-250
1977
ISSN/ISBN: 0022-3476 PMID: 830917 Document Number: 116231
Patients with HED were evaluated for directional patterning of scalp hair. These included 4 personally examined unrelated boys with X-linked HED and 4 other patients with this disorder, 2 patients with autosomal recessive HED, and 1 with another variety of HED in which normal dentition and cataracts are features. None of the patients had any abnormality of brain size and/or shape as judged by appearance and/or measurements. Aberrant scalp hair patterning may not only be a clue to problems in early brain morphogenesis, but may also constitute a clue to early problems in the morphogenesis of skin and its derivatives, in this instance HED. This feature allows for the early suspicion of such an ectodermal dysplasia. Early detection of HED may assist in the recognition of hypohidrosis and the attendant risk of excessive hyperthermia.