Hyper-IgM syndrome is a rare immunodeficiency disease characterized by mark
edly decreased serum IgG, IgA, and IgE levels but normal or elevated IgM le
vels. The most common clinical signs are infections, cirrhosis, arthritis,
malignancies, and mucosal ulcers, Approximately two-thirds of patients have
chronic neutropenia associated with oral and perirectal ulcers, We report
a 14-month-old girl with hyper-IgM syndrome who has recurrent cutaneous ulc
ers restricted to the diaper area.