Perforation of the nasal wall and hyper-IgE syndrome

Hyper-IgE syndrome is basically characterized by recurrent infections, chronic eczematous lesions, specific IgE antibodies against Staphylococcus aureus and markedly high serum IgE values. We present the case of an 11-year-boy with no relevant personal or family history, who came to our Department with highly pruriginous papulovesicular skin lesions of 3 years' duration. He presented marked obesity (+4 SD) and micropapulovesicular lesions in the trunk and extension areas of the limbs. The rest of the physical exploration was normal. Complementary studies revealed peripheral eosinophilia, increase in globular sedimentation rate and IgE values of 20,000 IU/ml, a nonspecific reaction to skin tests, and a skin biopsy compatible with atopic dermatitis. Three months later, he presented eczematous lesions in the trunk and limbs, perforation of the nasal wall due to staphylococcal abscess (diagnosed by biopsy), bilateral maxillary sinusitis and IgE values of 59,238 IU/ml. The differential diagnoses are discussed, as well as new diagnostic-therapeutic possibilities.