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Vol. 14. Issue 3.
Pages 164-167 (May - June 2018)
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Vol. 14. Issue 3.
Pages 164-167 (May - June 2018)
Case Report
DOI: 10.1016/j.reumae.2016.11.004
Dacryoadenitis Associated With IgG4-related Disease in an Afro-Colombian Adolescent
Dacrioadenitis por enfermedad relacionada con IgG4 en una adolescente afrodescendiente de Colombia
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Gabriel Sebastián Díaz-Ramíreza, Luis Fernando Medina-Quinteroa,b, Alexander Salinas-Césara, Andrés Felipe Zea-Veraa,c,
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a Departamento de Medicina Interna, Universidad del Valle, Cali, Colombia
b Medicina Interna-Reumatología, Universidad del Valle, Cali, Colombia
c Departamento de Microbiología, Universidad del Valle, Cali, Colombia
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Abstract

IgG4-related disease (IgG4-RD) is a recently recognized clinical condition with multiple aspects not yet elucidated. It is characterized by a fibrous inflammatory process that involves multiple organs and clinical, serological and histopathological findings, which represent a major challenge for the clinician. Classically described as an expansive tumor lesion with storiform fibrosis, lymphoplasmacytic infiltration (IgG4-positive) and elevated serum IgG4. Clinical features are variable, and pancreatic as well as extrapancreatic involvement has been reported, more frequently in Asian men over 50 years and rarely described in black people. We report the case of an Afro-Colombian teenage woman, who had a unilateral ocular protrusion of unknown cause, with histopathologic findings that revealed infiltration of lymphocyte and plasma cells into the lacrimal gland. It was positive for IgG4, ruling out other conditions, and confirming IgG4-related dacryoadenitis.

Keywords:
Dacryoadenitis
IgG4-related disease
Adolescent
Resumen

La enfermedad relacionada con IgG4 (ER-IgG4) es una condición clínica recientemente reconocida, con múltiples aspectos aún no dilucidados. Se caracteriza por el compromiso fibroinflamatorio de múltiples órganos; con hallazgos clínicos, serológicos e histopatológicos que representa un importante reto para el clínico. Clásicamente descrita como una lesión tumoral expansiva con fibrosis estoriforme, infiltración linfoplasmocítica (IgG4 positiva) e IgG4 sérica elevada. Las características clínicas son variables, se describe tanto compromiso pancreático como extrapancreático, es de predominio en varones asiáticos mayores de 50 años, y rara vez es descrita en personas de raza negra. Presentamos el caso de una mujer, adolescente, afro-colombiana, que presenta protrusión ocular unilateral inexplicable, con hallazgos histopatológicos que revelan infiltración de células linfocíticas y plasmáticas en la glándula lacrimal, con positividad para IgG4, descartándose otras condiciones, lo que confirma una dacrioadenitis por enfermedad relacionada con IgG4.

Palabras clave:
Dacrioadenitis
Enfermedad relacionada con IgG4
Adolescente

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