Scleroma; review of literature and first report of multiple familial occurrence in the United States.

SCLEROMA is a specific chronic granuloma localized in the rhinopharyngeal tract, involving the tissues of the nose, pharynx, larynx, trachea and bronchi. The disease was first described in 1870 by von Hebra,1a Viennese dermatologist. It undoubtedly had existed in eastern Europe for many years prior to that time, as evidenced by the fact that wax models of this disease made in 1840 were on exhibit at the Museum of the History of Medicine in Cracow, Poland. These had been presented by Bierkowsky2as examples of carcinoma. Von Hebra himself expressed the belief that this condition, which he termed rhinoscleroma (hard nose), was a granulomatous sarcoma or an atypical form of syphilis. Geber,3von Hebra's assistant, stated the opinion that it was a specific entity. In 1876, Mikulicz4reported the presence of peculiar foam cells seen in histologic studies. In the same year Gerhardt5first