A case of pain abdomen with skin rash

Abstract

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.