Sindrome Hiper Ige

HYPER IG E SYNDROME (HIES) VS SEVERE ATOPIC DERMATITIS
MILLA M, PEREZ J. ALMENARA HOSPITAL
INTRODUCTION
Hyper-IgE syndrome (HIES) is a rare primary immunodeficiency (PID). The staphylococcal skininfections recurring, recurrent pneumonia and high levels of IgE constitute the Clinic Triad of SHIE. There are two types: dominant autosomal (AD HIES) and recessive autosomal (AR HIES), that can bedistinguished by certain clinical findings (TABLE 1). 1-5. Recently a STAT3 mutation has been described as origin of dominant autosomal hyperimmunoglobulin E syndrome.6
REPORT OF A CASE
A13-year-old woman patient with a lifelong history of asthma, allergic rhinitis and severe atopic dermatitis was admitted to the Dermatology Service because of recurrent staphylococcal skin infections (2episodes of furunculosis and 3 of cellulitis), difficulties controlling his asthma and eczema. She had history of Pneumonias recurring without forming cysts or pneumatoceles (4 episode), recurrent urinaryinfections, retention of primary teeth and need for extraction.
She hasn´t consanguineous parents but a great-grandmother had atopic dermatitis and a maternal aunt with Lupus. She presented IgEvalues of up to 20,350 mU/ (Figure 1), eosinophils count was 580 cells/mm3, IgG, 908 mg/dl (normal: 700 - 1600 mg/dl), IgM, 130 gr/ml (normal: 40 – 230 mg/dl); IgA 168 mg/dl (normal: 70 – 400 mg/dl),IgE specific : Derm. Pteronyssinus 21.4 U/ml, Derm. Farinae 10,5 U/ml, Derm. Microceras 7,7 U/ml, leche 1.28 U/ml and positive culture at St aureus. The Hyper-IgE Syndrome Scoring Sheet was 38(Table 2).
TABLE1.Clinical findings of AD and AR HIES
TABLE 2. Hyper-IgE Syndrome Scoring Sheet.

DISCUSION:
Is this case a HIES or is it only atopia? The HIES scoring system uses at least 40points as a cutoff for a diagnosis of HIES.7 The scoring sheet of this patient is indeterminate; Patients with certain conditions such as severe atopic disease may occasionally have very high IgE...