IGHE and immune system disorder: However, general practitioners, pediatricians, pulmonologists, and dermatologists who are not aware of primary immunodeficiencies often still do not recognize the pathognomonic signs of AD-HIES (Fig. 4) and often misdiagnose elevated IgE and eczema as signs of allergy or chronic urticaria, even if there is a remarkable medical history for recurrent infections, even as severe as skin abscesses and pneumatoceles (Fig. 3).