A case of pain abdomen with skin rash

12 year old girl with history suggestive of chronic dermatitis from 3 months age and past pulmonary tuberculosis presented with one year history of intermittent, central abdominal pain with weight loss. She had coarse facial features with broad nasal bridge, eczematous skin rash, and dystrophic nails and on evaluation was found to have peripheral eosinophilia, very high IgE levels, imaging showed Bronchiectasis and pulmonary nodules. Eosinophilic enterocolitis was established on intestinal biopsies with exclusion of parasitic infestation and marrow pathology.Hyper-immunoglobulin -E syndrome is characterized by eczema -like skin rashes, recurrent staphylococcal infections, severe lung infections leading to pneumatoceles and very high levels of serum antibody IgE-usually 2000 IUmL. It could be inherited in autosomal dominant or recessive pattern or it may be of sporadic form. They are predisposed to recurrent fractures and skeletal deformities like scoliosis.This patient fulfilled the criteria for Hyper IgE syndrome. The pain was explainable with the eosinophilic enterocolitis. She responded partially to antihistaminics, antileukotriene agent-Monteleukast and dietary measures of excluding wheat, egg, corn, milk, seafood and groundnut. This combination of chronic eczema with HyperIgE and eosinophilic enterocolitis is very rare.