Premium
Cutaneous Sarcoid‐like Granulomas in a Patient with X‐Linked Hyper‐IgM Syndrome
Author(s) -
Gallerani Isabella,
Innocenti Damiano Degli,
Coronella Giordana,
Berti Samantha,
Amato Laura,
Moretti Silvia,
Fabbri Paolo
Publication year - 2004
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 - 10.1111/j.0736-8046.2004.21107.x
Subject(s) - medicine , buttocks , hypogammaglobulinemia , pathology , lesion , dermatology , gamma globulin , immunoglobulin m , immunoglobulin g , antibody , immunology , surgery
We describe a 5‐year‐old boy with red‐pink, firm, nodular lesions, with central resolution and prominent borders, localized to the face, backs of the hands, extensor surfaces of the arms and legs, and the buttocks. He also had recurrent bacterial respiratory infections. Quantitative immunoglobulin levels revealed hypogammaglobulinemia and increased IgM levels. Histopathologic examination of a nodular lesion revealed perivascular and periadnexal granulomas composed of epithelioid cells surrounded by a mantle of lymphocytes; three cultures for fungi and acid‐fast bacilli were negative. Clinical, histopathologic, and immunologic studies supported a diagnosis of hyper‐IgM (HIM) syndrome. The boy was treated with pulsed‐dose antibiotics and intravenous gamma globulin every 3 weeks, with improvement of clinical symptoms. Skin lesions were treated with topical corticosteroids, with immediate recurrence upon cessation of therapy. To the best of our knowledge, this is the first description of cutaneous granulomas in HIM syndrome.