Abstract

IgG4-related disorder (IgG4-RD) is a recently defined systemic disorder characterized by a unique fibro-inflammatory response that involves multiple organs including lymph nodes, salivary, lacrimal glands, and the pancreas as well as other endocrine organs. These organs display lymphoplasmacytic infiltrate with positive staining for IgG4 and high levels of circulating IgG4. We present an unusual case of IgG4-RD in a 55-year-old African-American female, who presented with an isolated chronic indurated painful mass in the thigh with a normal IgG4. Increased awareness with variable manifestations of IgG4-RD will facilitate the diagnosis of this condition. J Hematol. 2016;5(3):113-115 doi: http://dx.doi.org/10.14740/jh287w

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