Panel A shows table summarizing clinical characteristics and disorders across ten patients. Panel B shows scatter plot of eosinophil counts across different patient ages. Panel C shows scatter plot of immunoglobulin E concentrations across patient age groups. Panel D shows clinical photographs demonstrating severe atopic dermatitis manifestations in affected patients. Panel E shows photographs illustrating skin improvement following dupilumab treatment in Patient 1.
Major clinical features of the 10 patients. (A) Tabulation and comparison of the clinical phenotype for 10 patients. *Please note that for P3, we had limited information about his family pedigree or childhood growth trajectory. (B) Eosinophil count in whole blood. The shaded area represents counts <0.5 × 109/liter, which is the typical upper limit of normal. The horizontal broken line denotes an eosinophil count of 1.5 × 109/liter, since hypereosinophilic syndrome is traditionally defined as sustained peripheral blood eosinophilia >1.5 × 109/liter. (C) IgE concentration in whole blood. The shaded area represents IgE <240 μg/l, which is the typical upper limit of normal. (D) Photographs of widespread and severe atopic dermatitis in P1 and P4 during infancy. Photograph of P10’s facial skin, which shows extensive acute dermatitis with periorbital swelling. (E) Photographs of P1’s skin, which dramatically improved after dupilumab was started at 4.5 years of age.