Mucocutaneous manifestations of the hyper-IgM immunodeficiency syndrome

The recurrent pyogenic infections of patients with hyper-IgM syndrome are controlled by intravenous gamma globulin administration, but patients may suffer from early-onset oral ulcerations and warts.

We have characterized the mucocutaneous manifestations associated with this condition to allow physicians to more readily identify it.

Three male patients with the mucocutaneous manifestations of the hyper-IgM syndrome are described. In one, histopathologic examination of the oral mucosal lesion was performed.

Recurrent 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 ulcer showed a heavy infiltrate of mixed inflammatory cells. Warts tend to be widespread and resistant to traditional therapy.

Physicians should consider this uncommon condition when examining a male patient with severe oral ulcers or recalcitrant widespread warts.