Generalized Hair-Follicle Hamartoma

Abstract

An unusual syndrome of progressive baldness resulting from a distinctive hamartoma of the hair follicle is presented. The patient also had myasthenia gravis, an enlarged sella turcica, and an abnormal urinary amino acid pattern and was suspected of having lupus erythematosus. The generalized hair loss was diagnosed as alopecia universalis until multiple skin biopsies revealed a basal cell hamartoma of each hair follicle. Histochemically and by electron microscopic studies the tumor had characteristics of epithelial cells. A wax reconstruction showed the normal anatomic relationship of sebaceous glands and the arrectores pilorum muscles to this tumor. This study emphasizes the importance of skin biopsy in alopecia of unknown cause and suggests that further study of abnormal ectodermal tissues might provide insight into hair keratin defects.