“…All inherited forms of HIES express elevated IgE. Distinguishing features associated with AD-HIES are connective tissue and skeletal abnormalities to include maxillofacial irregularities and a prominent arched palate, recurrent skin and pulmonary infections (eczema, pneumatoceles), recurrent fungal infections, prolonged healing of infections, retained and/ or delayed shedding of primary teeth, and ligneous gingivitis/periodontitis (destructive membranous periodontal disease) (Hatori et al, 1993;Gunhan et al, 1994Gunhan et al, , 1999Grimbacher et al, 1999;Aldous et al, 2007;Freeman et al, 2009;Deepa et al, 2012;Sivolella et al, 2012). Grimbacher et al revealed that over 70% of their studied patient population with HIES had retained primary dentition (Grimbacher et al, 1999).…”