[Cryomacroglobulinemic purpura with liver cirrhosis and hepatic porphyria].
Hepatic porphoria and histologically verified cirrhosis, teleangiectasia, purpose, papular skin lesions existing for eight years, as well as cryomacroglobulin were found in a 49 year old man. Pathogenetically the liver disease is considered to be the primary event, which was accompanied by secondary cryoglobulinemia. IgG and IgM type of thrombus in the vessels and of small granular deposits corresponding to the type of cryoprotein in the blood, were detected in the vessel walls of the upper part in the dermis and along these vessels of both purpuri and papular skin lesions. Immunoprecipitates could be revealed by FTC-labelled antihuman complement. In the skin lesions porphyrin could not be demonstrated. The investigation of cryoglobulin and of porphyrin disorders is reasonable in every patient with purpura accompanying pathological liver processes.
D003450:Cryoglobulins / Q000032:analysis; D005455:Fluorescent Antibody Technique; D006801:Humans; D007074:Immunoglobulin G; D007075:Immunoglobulin M; D007136:Immunoglobulins / Q000032:analysis; D008103:Liver Cirrhosis / Q000150:complications*; D008214:Lymphocytes; D008297:Male; D008875:Middle Aged; D011164:Porphyrias / Q000150:complications* / Q000652:urine; D011166:Porphyrins / Q000032:analysis / Q000652:urine; D011694:Purpura, Hyperglobulinemic / Q000150:complications* / Q000473:pathology; D012867:Skin / Q000032:analysis / Q000473:pathology
D003450:Cryoglobulins; D007074:Immunoglobulin G; D007075:Immunoglobulin M; D007136:Immunoglobulins; D011166:Porphyrins
Purpura cryomacroglobulinaemica bei mit Leberzirrhose einhergehender hepatischer Porphyrie.