Granulomatose dewegener e hanseníase: apenas uma associação fortuita?

Raul Negrão Fleury; Paulo Rogério O. Taborda; Diltor Vladimir Araújo Opromolla

doi:10.47878/hi.1996.v21.36477

Authors

Raul Negrão Fleury Chefe da Equipe Técnica de Clínica e Terapêutica do Instituto "Lauro de Souza Lima" - Bauru/SP
Paulo Rogério O. Taborda Médico Dermatologista do Instituto "Lauro de Souza Lima"
Diltor Vladimir Araújo Opromolla Diretor da Divisão de Pesquisa e Ensino do Instituto "Lauro de Souza Lima" - Bauru/SP

DOI:

https://doi.org/10.47878/hi.1996.v21.36477

Keywords:

Wegener's granulomatosis, complication, leprosy

Abstract

The authors review a case of a male patient presenting borderline lepromatous leprosy for 28 years. After release from treatment ( with negative skin-smears) he was under irregular dapsone monotherapy. Ten years later he showed again active skin lesions with positive skin smears (2+ with some solid stained bacilly ). Suspecting of dapsone resistance, he was put under Clofazimine for 12 months showing partial regression of the skin lesions. Although he continued to use dapsone after this period, the skin lesions contined to show regression and skin smears presented no solid stained bacilli. Episodes of ENL were note in this period. Four years before death, he began to present signs of Chronic Renal Failure which was
related to a probable secondary amyloidosis due to leprosy. Autopsy revealed (1) intense impregnation of CFZin the mucosa of the upper air up-pathway stomach and bowels, as well as in limphnodes; (2) regressive lepromatous infiltrate with positive baciloscopy with granular bacilli in various sites and some organs sucha as liver, spleen and limphnodes; (3) amyloid deposits in several organs and (4) a necrotizing nodular lesion close to the principal bronchi of the right lung, showing palisaded granuloma and granulomatous vasculitis, which was interpreted as a localized pulmonary maniíestation of Wegener's Granulomatosis (WG). The evidences showed that it was indeed a case of advanced borderline leprosy associated, fortuitously or not, to a WG, in the initial phase or an asymptomatic limited form. There is no mention of such association in the literature. It is assumed that the WG could be related to some unidentified bacteria which exposes neutrophilic proteasis producing antibodies to neutrophil cytoplasmic antigens (ANCAs). The authors suggest the possibility of leprosy, to be the underlying bacterial disease in this case. The
determination of these antibodies in leprosy and the search for WG may suggest a real association between these two intriguing clinical entities.

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