CUTANEOUS HISTOPLASMOSIS IN A CHILD WITH HYPER-IGM

Immunodeficiency with hyperimmunoglobulinemia M is a rare disease char acterized by very low levels of IgG and IgA and normal or high levels of serum IgM and IgD. Recurrent and severe systemic infections with pa thogenic bacteria are frequent if immunoglobulin replacement therapy i s not given. Histoplasmosis is a systemic granulomatous mycosis due to Histoplasma capsulatum and characterized by a particular affinity for the reticuloendothelial system. Glabrous skin involvement in histopla smosis is highly unusual except in patients with advanced human immuno deficiency viral disease. Cutaneous histoplasmosis and granulomatous r eaction were diagnosed in a 5-year-old boy with hyper-IgM disease. The lesion improved after oral ketoconazole therapy. To our knowledge, th is is the first case of cutaneous histoplasmosis associated with hyper -IgM to be reported.