tailieunhanh - Báo cáo y học: "Erythropoietic Protoporphyria Masquerading as Angioedema in a 4-Year-Old Female"

Tuyển tập các báo cáo nghiên cứu về y học được đăng trên tạp chí y học General Psychiatry cung cấp cho các bạn kiến thức về ngành y đề tài: Erythropoietic Protoporphyria Masquerading as Angioedema in a 4-Year-Old Female. | Original Article Erythropoietic Protoporphyria Masquerading as Angioedema in a 4-Year-Old Female Helen C. Wang MD Ejaz Yousef MD Abstract Angioedema is a common presentation with a broad differential including rare disorders with which an allergist must be familiar. Our objective was to report a case of swelling of the hands and feet mimicking angioedema with hepatomegaly in a 4-year-old girl. The patient was evaluated for painful swelling of the hands and feet after exposure to sun. Examination revealed edema and erythema of the extremities and hepatomegaly. Laboratory evaluation included elevated liver transaminases and plasma protoporphyrin with normal urine porphyrins. Liver biopsy confirmed the diagnosis of erythropoietic protoporphyria a disorder of heme biosynthesis in which patients may present with photosensitivity and angioedema. It is important for allergists to recognize this entity in patients with cutaneous disorders of unclear etiology in order to prevent possible life-threatening sequelae. Angioedema is a common presenting symptom among allergy practices. Angioedema is caused by increased vascular permeability in the subcutaneous tissue of the skin and respiratory and gastrointestinal Acute angioedema is most often due to an immunoglobulin E IgE -mediated response to an inciting allergen and is classified as a type I hypersensitivity However there is a broad differential for angioedema including hereditary and acquired disorders. One rare diagnosis that occurs in the pediatric population and that allergists must be familiar with is angioedema-like cutaneous findings due to pho- . Wang E. Yousef Division of Allergy and Immunology Alfred I. duPont Hospital for Children Nemours Children s Clinic Wilmington Delaware Thomas Jefferson University Philadelphia Pennsylvania Correspondence to Dr. Ejaz Yousef Division of Allergy and Immunology Alfred I. duPont Hospital for Children 1600 Rockland Road Wilmington DE E-mail .