Abstract

PEREZ-GOELKEL, Snayder José; CHAO-PEREIRA, Caridad; VALDES-ALVAREZ, Karen  and  CAMPOS-GARCIA, María Paula. Paraneoplastic pemphigus as an atypical presentation of Castleman disease. Acta Med Colomb [online]. 2021, vol.46, n.2, pp.46-50.  Epub Nov 22, 2021. ISSN 0120-2448.  https://doi.org/10.36104/amc.2021.1964.

Castleman disease is a non-clonal lymphoproliferative disorder with a broad range of clinical manifestations. We present the case of a male patient with a clinical picture of asthenia, adynamia, hyporexia, weight loss, oral and genital ulcers, and red, itchy eyes. The physical exam showed conjunctival redness and oral and genital ulcers. Computed axial tomography with contrast of the chest and abdomen revealed multiple enlarged mediastinal and retroperitoneal lymph nodes, and a solid 94x51 mm retroperitoneal mass. A biopsy of the mass was taken, which reported the hyaline vascular variant of Castleman disease. A scrotal lesion biopsy was also ordered, with a histopathological analysis compatible with pemphigus. In addition, direct immunofluorescence was positive in the epidermal intercelullar spaces, as well as immunoprecipitation with anti-desmoglein, anti-desmoplakin, anti-envoplakin and pemphigoid ampule antigen. Thus, the presence of multicentric Castleman disease associated with paraneoplastic pemphigus was established. (Acta Med Colomb 2021; 46. DOI: https://doi.org/10.36104/amc.2021.1964)

Keywords : pemphigus; paraneoplastic; Castleman; multicentric.

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