SCLEREDEMA ADULTORUM BUSCHKE - CASE-REPOR T AND REVIEW OF THE LITERATURE

Scleredema adultorum of Buschke is a rare disorder of unknown aetiolog y. It is characterized by diffuse, non-pitting swelling and induration of the skin. Skin biopsies reveal marked thickening of the dermis due to collagenous replacement of the subcutis and deposition of hyaluron ic acid between the collagen fibers. The disease classically only affe cts the skin. In 24 cases an associated monoclonal gammopathy has been detected. A 75-year old patient had a 19 year history of scleredema a dultorum. In addition to a monoclonal gammopathy the patient suffered from involvement of the tongue, pharynx and upper esophagus. Furthermo re a polyneuropathy, ocular involvement with restricted eye movements and a sicca syndrome were present. The simultaneous occurrence of cuta neous scleredema with any one of the above mentioned symptoms has been reported before. The wide variety of extracutaneous manifestations of scleredema as found in our patient is amazing and has not been previo usly described.