LIPID PROTEINOSIS (URBACH-WIETHE)

Abstract

Though rare, the clinical picture of lipid proteinosis (Urbach-Wiethe disease) presents sufficient characteristics which cannot fail to be observed. Hoarseness and dyspnea due to laryngostenosis led laryngologists, above all, to take interest in the matter. This subject may be divided into four historical stages. The first refers to former indeterminate observations. Here one may include the case published successively by the laryngologist Siebenmann (1908), by the dermatologist Lutz (1922) and by the anatomopathologist Rössle (1927) with designations of "generalized hyperkeratosis of the skin with involvement of mucosa," "naevus icthyosiformis" and "hereditary progressive pachydermic dystrophy" respectively. In addition to this case, followed for nineteen years by several observers, there is a small group, 3 or 4 cases (1 of these of doubtful retrospective diagnosis), studied by Miescher and later by Nager (1928) under the names "familial keratosis of the skin and mucosae combined with bullous formations and colloidal degeneration of the