Hereditary Hypotrichosis and Localized Morphea: A New Clinical Entity

The document from 1986 describes a study of a family with a new clinical entity characterized by hereditary hypotrichosis (permanent hair loss) and localized morphea (a skin condition), suggesting an autosomal dominant inheritance pattern. The study involved three family members across three generations, including a 14-year-old girl, her father, and grandmother, all presenting with hair loss in childhood and skin lesions consistent with morphea. The father's skin biopsy supported a morphea diagnosis but was not conclusive. The pattern of hair loss was atypical for alopecia areata, with scalp hair remaining and other body hair loss being permanent. Additionally, the father exhibited ocular changes, which are not typical of alopecia areata. The researchers propose that the observed conditions might be related to a single genetic disorder and recommend further observation of hair growth patterns in familial scleroderma cases to identify other affected families. The study was funded by NIH Research Program project number AM 21557-09 and NIH training grant 07019(PK).