A 36-year-old pregnant woman with hyperimmunoglobulinemia E

Hyper-IgE syndrome (HIES) is an extremely rare multisystem disorder characterized by eczematoid dermatitis, recurrent staphylococcal cutaneous infections, recurrent pulmonary infections and elevated serum levels of IgE [1–2]. Characteristic facial appearance typically emerges in late childhood or early adolescence, including asymmetry, increased interalar distance, prominent forehead, deep-set eyes, broad nasal bridge, rough facial skin, prognathism, and high-arched palate [3]. In the course of the disease musculoskeletal, vascular and ocular abnormalities may also occur. The first signs of the disease may appear already in the newborn, but overall, the symptoms occur in late childhood or even early adolescence [1–3] © Copyright 2022 PTGiP