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June 1987

Hair Protein Patterns in a New Autosomal Dominant Ectodermal Dysplasia

Author Affiliations
Department of Dermatology Box 98, University Hospital 420 Delaware St SE; Department of Pediatrics Box 75, University Hospital 420 Delaware St SE; Dight Laboratories 214 Botany Bldg 400 Church St SE; Department of Laboratory Medicine and Pathology Box 198, University Hospital 420 Delaware St SE; School of Dentistry Division of Oral Genetics 16-262 Moos Health Sciences Tower 515 Delaware St SE; Dight Laboratories 214 Botany Bldg 400 Church St SE University of Minnesota Minneapolis, MN 55455
Arch Dermatol. 1987;123(6):716-718. doi:10.1001/archderm.1987.01660300032009

To the Editor.—  The ectodermal dysplasias form a heterogeneous group of conditions that are poorly understood both clinically and causally.1 We describe a new type of ectodermal dysplasia characterized by onychodysplasia and trichodysplasia that affected seven individuals spanning three generations. We attempted to find a hair protein alteration in this family that could aid in diagnosis and counseling. Hair samples were obtained from the unaffected mother and daughter and from the affected father and daughter in the first generation. These hairs were solubilized and hair proteins analyzed by sodium dodecyl sulfate polyacrylamide gel electrophoresis. A low-molecular-weight protein was found in the insoluble hair fractions obtained from the affected father and daughter, but not in those obtained from the normal family members or from a control group of normal individuals. The results of this family study suggest that hair proteins may be altered in the ectodermal dysplasias in which trichodysplasia is a prominent feature.