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An Unusual Presentation of Immunodeficiency with Hyper‐IgM
Author(s) -
Uğuz Ayşen,
Yilmaz Ertan,
Çiftçioğlu M. Akif,
Yeğin Olcay
Publication year - 2001
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1046/j.1525-1470.2001.018001048.x
Subject(s) - medicine , primary immunodeficiency , immunodeficiency , immunology , immunoglobulin e , neutropenia , immunoglobulin m , arthritis , dermatology , antibody , immunoglobulin g , immune system , chemotherapy
Abstract: Hyper‐IgM syndrome is a rare immunodeficiency disease characterized by markedly decreased serum IgG, IgA, and IgE levels but normal or elevated IgM levels. The most common clinical signs are infections, cirrhosis, arthritis, malignancies, and mucosal ulcers. Approximately two‐thirds of patients have chronic neutropenia associated with oral and perirectal ulcers. We report a 14‐month‐old girl with hyper‐IgM syndrome who has recurrent cutaneous ulcers restricted to the diaper area.