Generalized Hair-Follicle Hamartoma

The document described a unique syndrome characterized by progressive baldness due to a distinctive hamartoma of the hair follicle. The patient exhibited symptoms including myasthenia gravis, an enlarged sella turcica, and an abnormal urinary amino acid pattern, with a suspicion of lupus erythematosus. Initially diagnosed as alopecia universalis, multiple skin biopsies later identified a basal cell hamartoma in each hair follicle. Histochemical and electron microscopic analyses revealed the tumor's epithelial cell characteristics. The study highlighted the critical role of skin biopsy in diagnosing alopecia of unknown origin and suggested that further research into abnormal ectodermal tissues could enhance understanding of hair keratin defects.