First known report of food hypersensitivity or atopy in a person with the Prader-Willi syndrome

Abstract

Rationale We report the first known case of food allergy or atopy in the Prader-Willi Syndrome (PWS). Methods A case report followed by a summary of the literature. Results At 17 months of age our patient developed urticaria after eating egg on three occasions, with one episode of hoarseness. He had a strongly positive immediate egg skin test. He underwent a successful egg elimination diet, made difficult by the marked hyperphagia characteristic of the syndrome. A serum IgE Immunocap was negative to egg mix at age 41 months. He subsequently had a positive food challenge to egg at age 44 months with urticaria. PWS has an incidence of one in 10-15,000 live births. Affected persons develop hyperphagia at 1 to 6 years of age, with extreme food seeking behavior. Prader-Willi is caused by an alteration of the chromosomal material in q11-13 of chromosome 15. There are no reported cases of food allergy or urticaria in PWS. There are also no reported cases of anaphylaxis, atopic dermatitis, allergic rhinitis, or asthma in persons with this syndrome. Medline searches were done combining these conditions from 1966-present. Conclusions We report the first case of food allergy or atopy in PWS. Food allergy and atopy may be less common in PWS than the general population since there are no other cases in the literature. The hyperphagia characteristic of this syndrome makes a strict food elimination diet difficult. As this boy matures, the food seeking behaviors associated with this syndrome might place him at serious risk of anaphylaxis.