MUCOCUTANEOUS MANIFESTATIONS OF THE HYPER-IGM IMMUNODEFICIENCY SYNDROME

Background: The recurrent pyogenic infections of patients with hyper-I gM syndrome are controlled by intravenous gamma globulin administratio n, but patients may suffer from early-onset oral ulcerations and warts . Objective: We have characterized the mucocutaneous manifestations as sociated with this condition to allow physicians to more readily ident ify it. Methods: Three male patients with the mucocutaneous manifestat ions of the hyper-IgM syndrome are described. In one, histopathologic examination of the oral mucosal lesion was performed. Results: Recurre nt large, painful oral ulcerations can occur that are not necessarily associated with neutropenia nor do they respond to granulocyte colony- stimulating factor administration. Histopathologic examination of an u lcer showed a heavy infiltrate of mixed inflammatory cells. Warts tend to be widespread and resistant to traditional therapy. Conclusion: Ph ysicians should consider this uncommon condition when examining a male patient with severe oral ulcers or recalcitrant widespread warts.