LIPOIDOSIS cutis et mucosae has been reported so infrequently as to be considered a rare disease. However, sufficient cases1 have been presented in the literature so that it emerges as a well defined clinical entity with a pathognomonic histopathologic picture. According to Urbach,2 the disease is characterized by abnormal deposits of lipids in the skin, mucous membranes and possibly also in other tissues. From histochemical studies employing differential staining procedures and solubilities in various organic solvents, Urbach came to the conclusion that the lipid was of the group of acetone-soluble phosphatides and that it was combined with protein in a physical adsorptive union. Therefore, he suggested the name lipoid proteinosis for this disease.3 In support of this concept, Montgomery and Havens4 and J. Ramos e Silva5 were able to show a decided increase in the phospholipid content of lesions which were subjected to chemical analysis.