An Appraisal of Hyalinosis Cutis et Mucosae

Abstract

Hyalinosis cutis et mucosae is a rare disease which is recognized by dermatologists and some laryngologists but apparently not well known by physicians in other specialties. Hoarseness and dyspnea caused laryngologists to become interested in the disorder. The first case was reported by Siebenmann (1908) as "generalized hyperkeratosis of the skin with involvement of mucosa." Lutz (1922) and Rössle (1927) designated similar cases as "naevus ichthyosiformis" and "hereditary progressive pachydermic dystrophy." Wiethe (1924, 1927) reported six cases. Urbach (1929) stated that the disease was a disturbance of lipoid metabolism and named it "lipoidosis cutis et mucosae." He subsequently (1932) changed this appellation to "lipoid proteinosis." Tripp (1936) reported the first case in the United States, followed by Wise and Rein (1938), Montgomery and Havens (1939), Wile and Snow (1941), Sulzberger (1942), Ramos e Silva (1943), Price and his associates (1947), Lower (1948), Thannhauser (1949), and