Diagnosis of HyperIgE Syndromes (HIES) on Black Phenotype Patients with Atopic Dermatitis Followed in Dakar, Senegal

Abstract

This study aimed to determine the epidemiological and clinical-biological manifestation of hyper IgE syndromes during atopic dermatitis in the pediatric dermatology department of the Centre Hospitalier National d’Enfants Albert Royer (CHNEAR) on black phenotype children in Dakar, Senegal. It was a descriptive cohort study including patients with atopic dermatitis who consulted in Hôpital d’Enfants Albert Royer from 1st Janvier 2020 to 31st August 2022. We considered hyper IgE syndrome as any patient with atopic dermatitis, recurrent infections, elevated serum IgE and a National Institute of Health (NIH) score for the diagnosis of hyper IgE syndromes greater than or equal to 20. We collected 27 cases accounting for a frequency of 0.78% among 3438 cases of atopic dermatitis. The mean age was 7 years with a sex ratio of 02. Consanguinity was found in 33.3% of patients. Cutaneous infections were found in 81.5% of cases. Bacterial infections were found in 66.6%. Viral and fungal infections stood for 11.1% and 29.6% respectively. Extracutaneous manifestations were infectious in 29.6% and non-infectious, consisting of facial dysmorphia (85.2%), scoliosis (n=01), joint hyper-extensibility (n=01) and retention of primary teeth (n=01). The mean total IgE level was 6094.7 KIU/L. Hypereosinophilia was found in 63%. The NHIES score was between 20 and 40 in 96.3% and above 40 in 3.7%. The frequency of hyper IgE syndromes in atopic dermatitis is 0.78%. Bacterial infections predominate. A genetic testing is necessary to better describe these HIES.