Yilmaz G G, Yilmaz E, Coşkun M, Karpuzoğlu G, Gelen T, Yeğin O
Department of Pediatrics, Akdeniz University, Medical Faculty, Antalya, Turkey.
Pediatr Dermatol. 1995 Sep;12(3):235-8. doi: 10.1111/j.1525-1470.1995.tb00166.x.
Immunodeficiency with hyperimmunoglobulinemia M is a rare disease characterized by very low levels of IgG and IgA and normal or high levels of serum IgM and IgD. Recurrent and severe systemic infections with pathogenic bacteria are frequent if immunoglobulin replacement therapy is 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 histoplasmosis is highly unusual except in patients with advanced human immunodeficiency viral disease. Cutaneous histoplasmosis and granulomatous reaction were diagnosed in a 5-year-old boy with hyper-IgM disease. The lesion improved after oral ketoconazole therapy. To our knowledge, this is the first case of cutaneous histoplasmosis associated with hyper-IgM to be reported.
伴高免疫球蛋白M的免疫缺陷是一种罕见疾病，其特征为IgG和IgA水平极低，血清IgM和IgD水平正常或升高。如果不进行免疫球蛋白替代治疗，常反复发生由病原菌引起的严重全身感染。组织胞浆菌病是由荚膜组织胞浆菌引起的系统性肉芽肿性真菌病，其特点是对网状内皮系统有特殊亲和力。除晚期人类免疫缺陷病毒病患者外，组织胞浆菌病累及无毛皮肤非常罕见。一名患有高IgM疾病的5岁男孩被诊断为皮肤组织胞浆菌病和肉芽肿反应。口服酮康唑治疗后病变好转。据我们所知，这是首例报道的与高IgM相关的皮肤组织胞浆菌病病例。
