Aftosis oral y laringitis, ¿otra forma de presentación de la enfermedad relacionada con IgG4? / Aphthous stomatitis and laryngitis, another form of presentation of an IgG4-related disease?

La enfermedad relacionada con IgG4 (ER-IgG4) se caracteriza por un infiltrado linfoplasmocítico rico en células plasmáticas IgG4 positivas, fibrosis estoriforme y flebitis obliterativa. Se puede presentar como seudotumor orbitario, parotidomegalia, nefritis túbulo intersticial, fibrosis retroperitoneal o pancreatitis, aunque prácticamente cualquier órgano puede verse afectado. Presentamos el caso de una mujer de 37 años, que presenta un cuadro de disfonía severa y aftosis oral dolorosa recurrente, con unos hallazgos histopatológicos a nivel laríngeo que muestran infiltrado linfoplasmocítico y positividad para IgG4, así como amplios estudios descartando otras etiologías, por lo que se confirma una laringitis por ER-IgG4, cuya descripción en la literatura es excepcional

IgG4-related disease is characterized by a lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis and obliterative phlebitis. It can present as parotid gland enlargement, tubulointerstitial nephritis, retroperitoneal fibrosis or pancreatitis, although nearly any organ can be affected. We report the case of a 37-year-old woman who presented with severe dysphonia and recurrent painful aphthous ulcers, with histopathological findings at the level of the larynx that revealed a lymphoplasmacytic infiltrate and IgG4 positivity. In addition, extensive studies were performed to rule out other diseases. Thus the diagnosis was IgG4-related laryngitis, an exceptional finding in the literature

Aphthous Stomatitis and Laryngitis, Another Form of Presentation of an IgG4-Related Disease? / Aftosis oral y laringitis, ¿otra forma de presentación de la enfermedad relacionada con IgG4?

IgG4-related disease is characterized by a lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis and obliterative phlebitis. It can present as parotid gland enlargement, tubulointerstitial nephritis, retroperitoneal fibrosis or pancreatitis, although nearly any organ can be affected. We report the case of a 37-year-old woman who presented with severe dysphonia and recurrent painful aphthous ulcers, with histopathological findings at the level of the larynx that revealed a lymphoplasmacytic infiltrate and IgG4 positivity. In addition, extensive studies were performed to rule out other diseases. Thus the diagnosis was IgG4-related laryngitis, an exceptional finding in the literature.