title: [Scleredema adultorum Buschke. Case report and review of the literature].
abstract: Scleredema adultorum of Buschke is a rare disorder of unknown aetiology. 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 hyaluronic acid between the collagen fibers. The disease classically only affects the skin. In 24 cases an associated monoclonal gammopathy has been detected. A 75-year-old patient had a 19-year history of scleredema adultorum. In addition to a monoclonal gammopathy the patient suffered from involvement of the tongue, pharynx and upper esophagus. Furthermore a polyneuropathy, ocular involvement with restricted eye movements and a sicca syndrome were present. The simultaneous occurrence of cutaneous 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 previously described.
authors.affiliation: Universitäts-Hautklinik Tübingen.
authors.forename: A
authors.initials: A
authors.lastname: Ulmer
authors.forename: G
authors.initials: G
authors.lastname: Schaumburg-Lever
authors.forename: J
authors.initials: J
authors.lastname: Bauer
authors.forename: I
authors.initials: I
authors.lastname: Kötter
authors.forename: G
authors.initials: G
authors.lastname: Fierlbeck
mesh_terms: D000368:Aged; D001706:Biopsy; D003094:Collagen / Q000378:metabolism; D006801:Humans; D006820:Hyaluronic Acid / Q000378:metabolism; D008297:Male; D010265:Paraproteinemias / Q000175:diagnosis / Q000473:pathology; D012592:Scleredema Adultorum / Q000175:diagnosis* / Q000473:pathology; D012867:Skin / Q000473:pathology
chemical_list: D006820:Hyaluronic Acid; D003094:Collagen
vernacular_title: Scleroedema adultorum Buschke. Fallbericht und Literaturübersicht.