高グロブリン血症性紫斑病—症例報告

PURPURA HYPERGLOBULINEMICA

はじめに

　高グロブリン血症性紫斑病^1）（purpura hyper-globulinemica，Waldenström）の記録はわが国に20例^2）〜21）みられるが，皮膚科からの報告はまだない。われわれの観察した本症の1例を述べ，若干の考察を加える。なお，本例は金沢大学皮膚科で扱われた患者であるが，血清蛋白の諸検査の一部については徳島大学第一内科（西条一夫博士）の援助を受けた。

　A 32-year-old housewife had been suffering from attacks of purpura since 20 years of age, which appeared especially after long walks. In 1962 the purpura was accompanied with arthralgia, epistaxis and gingival bleeding. She had been pointed the disturbance of the heart by a doctor since her childhood.

　On February 7, 1963, she visited at first the dermatologic clinic of the University Hospital. She had small petechiae and livedo reticularis on the lower legs.

　Histologic specimen from the purpura on the lower leg showed slight perivascular infiltration in the upper and middle dermis composed of neutrophils, lymphocytes and nuclear fragments. The vascular wall showed edematous swelling, degeneration and bleeding.

　Histologic specimen from the inguinal lymph node showed proliferation of reticulum cells, accompanied with many plasma cells in some parts and some melanophores.

　Laboratory tests revealed normal bleeding and coagulation time and normal number of platelets, while anemia and strongly positive Rumpel-Leede's test were proved. The results of liver function tests, E. S. R., and serum protein showed extreme abnormalities. No Bence-Jones' protein was proved in the urine. Immunological and chemical analysis of the serum showed total protein 9.4-10.3 g/dl, γ-globulin 43.6-50.0% (Tiselius) , increased 7S globulin with normal amount of 19S (ultracentrifugation), and increased amount of IgG (immunoelectrophoresis), increased serum viscosity, slightly increased amount of fibrinogen and antitripsin ; cryoglobulin (-) , cryofibrinogen (-), LE test (-) , CRP (-) and RA (⧺).