Abnormal neutrophil chemotaxis due to decreased production of interferon gamma by T lymphocytes is thought to cause the disease. Both autosomal dominant and recessive inheritance have been described: Autosomal dominant: * STAT3 may present as<unk>  with characteristic facial, dental, and skeletal abnormalities that has been called Job's Syndrome. A common mnemonic used to remember the symptoms is FATED: coarse or leonine facies, cold staph abscesses, retained primary teeth, increased IgE, and dermatologic problems eczema. The disease was linked to mutations in the STAT3 gene after cytokine profiles indicated alterations in the STAT3 pathway. This altered pathway directly reduces the modulation capacity of interleukins 6 and 10 which, respectively, inhibit the genesis of Th17 cells that, in tandem with CD4 cells, protect against bacterial and fungal infections, and foster the inappropriate immune responses exhibited by those with Job Syndrome. Autosomal recessive: * DOCK8 - DOCK8 Immunodeficiency Syndrome (DIDS) presents primarily with immune effects including HEIS. Eczema is prominent, food and environmental allergies are common, and asthma and anaphylaxis has been variably reported. * PGM3, a Congenital Disorder of Glycosylation, may present as<unk>  with neurocognitive impairment and hypomyelination. See PGM3 deficiency. * SPINK5 may present as<unk>  with skin and hair effects such as trichorrhexis invaginata (bamboo hair). See Netherton Syndrome (NTS). * TYK2 may present as<unk> , although more often only with immunodeficiency.
Hyperimmunoglobulin E syndrome
Anti-IgLON5 disease
Wiskott–Aldrich syndrome
MOG antibody disease
Hyper-IgM syndrome type 3
Hyper-IgM syndrome type 5
TNF receptor associated periodic syndrome
Hyper-IgM syndrome type 4