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February 1965

Picture of the Month

Am J Dis Child. 1965;109(2):147-148. doi:10.1001/archpedi.1965.02090020149010

Denouement and Discussion 

ECTODERMAL DYSPLASIA  I. ANHIDROTIC TYPEMajor ManifestationsAplasia or hypoplasia of sweat, sebaceous and mucous glands; elevated temperatures; abnormal, delayed or absent dentition; hypotrichosis.GeneticsSex-linked recessive which may be irregularly dominant.Sexual IncidenceOccurs mainly in males.SymptomatologyUnexplained fevers; decreased lacrimation, taste, and smell; chronic atrophic rhinitis, pharyngitis, and conjunctivitis, hoarse voice, and dysphagia.Physical FindingsProminent frontal bossing, saddle-shaped nose, protruding or deformed ears; eyelids are thin and wrinkled with a prominence of the supraorbital ridge; thick and protruding lips; delayed or absent dentition. When present, the incisors are widely spaced and the canines have a common conical morphology. The scalp hair is fine, sparse, and grows slowly. Body hair is very fine or absent. The skin is delicate, translucent, smooth, thin, dry, and may have lack of pigmentation. The mammary glands may be hypoplastic or absent.DiagnosisThere is absence or deficiency of

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