Wegener's Granulomatosis

WEGENER'S GRANULOMATOSIS

I．はじめに

　1936年，ついで1939年にF．Wegener^1）2）は気道，とくに上気道の進行性壊死性過程が先行し，全身に散布性病巣，とくに腎に障害のみられることの多い疾患について病理学的に検討し，壊死性鼻炎よりこれを分離，一独立疾患とした。その後欧米諸家の報告があいつぎ，その独立性が確認されたが，本邦でも1940年宮田^3）の報告以来，耳鼻科，内科，眼科領域で症例が多くみられるにいたつた。一方皮膚科領域ではその主たる病変が他科領域に属するためか，わずか5例の報告しかみていない。ここに自験例を報告し，あわせて集め得た本邦例について若干のかんがえを加えてみたい。

A case of Wegener's granulomatosis of a 34-year-old female was reported. It started about 6 years ago with symptoms of chronic sinusitis, the diagnosis of which was confirmed by biopsy from the inferior nasal concha. After that time symptoms and abnormal findings of the eyes, joints, urine and blood developed, indicating systemic involvement.

She was hospitalized on account of severe bleeding from the nose. Her face was edematous with saddle nose. Many blisters on the scalp, forehead, face, retroauricular regions, forearms and backs of both hands and necrotic papules with crusts on the dorsal surface of the fingers, legs, ankles, patellar and cubital regions were noted.

Histologic specimens of the inferior nasal concha showed giant cell granuloma and those of a vesicle on the forearm showed necrotizing angiitis.

Statistic studies on reported 74 cases in Japan including the author's case were performed. No sex difference in frequency of incidence and the highest occurence in the third decade of life were noted. Most of the cases had triad of the nose, eye and kindney, and main skin manifesta-tions were ulcer, erythema, ecchymosis and bulla on the exposed areas. Most of the patients died about one year after the disease fully developed.