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April 1971

Hereditary Acrokeratotic Poikiloderma

Author Affiliations

Charlottesville, Va; Roanoke, Va; Wilson, NC

From the Department of Dermatology, University of Virginia School of Medicine, Charlottesville, Va (Dr. Weary); Roanoke, Va (Dr. Manley); and Wilson, NC (Dr. Graham).

Arch Dermatol. 1971;103(4):409-422. doi:10.1001/archderm.1971.04000160059010

A dominantly inherited disorder has affected ten members of a family group. At present we recognize the following categories of clinical involvement: (1) vesicopustule formation which remains confined to the hands and feet (this process commences at from 1 to 3 months of age and resolves in late childhood); (2) widespread eczematous dermatitis somewhat resembling atopic eczema which starts between the ages of 3 and 6 months and completely resolves by 5 years of age; (3) the gradual appearance of a diffuse poikiloderma with striate and reticulate atrophy which spares only the face, scalp, and ears (the poikiloderma persists into adult life); and (4) the development of keratotic papules on the hands, feet, elbows, and knees, which first appear at varying times prior to 5 years of age and persist indefinitely.

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