The cause of<unk>  is unknown. It is frequently discussed together with other<unk><unk>  syndromes. Possible causes include primarily genetic or due to an initial infection. The condition appears to be the result of a disturbance of innate immunity. The changes in the immune system are complex and include increased expression of complement related genes (C1QB, C2, SERPING1), interleukin-1-related genes (interleukin-1B, interleukin 1 RN, CASP1, interleukin 18 RAP) and interferon induced (AIM2, IP-10/CXCL10) genes. T cell associated genes (CD3, CD8B) are down regulated. Flares are accompanied by increased serum levels of activated T lymphocyte chemokines (IP-10/CXCL10, MIG/CXCL9), G-CSF and proinflammatory cytokines (interleukin 6, interleukin 18). Flares also manifest with a relative lymphopenia. Activated CD4(+)/CD25(+) T-lymphocyte counts correlated negatively with serum concentrations of IP-10/CXCL10, whereas CD4(+)/HLA-DR(+) T lymphocyte counts correlated positively with serum concentrations of the counterregulatory IL-1 receptor antagonist.
Periodic fever, aphthous stomatitis, pharyngitis and adenitis
Pruritic urticarial papules and plaques of pregnancy
Vesiculopustular eruption and leukemoid reaction in Down syndrome
Asymmetric periflexural exanthem of childhood
Photosensitivity with HIV infection
Chronic mucocutaneous candidiasis
Tubulointerstitial nephritis and uveitis
Multifocal choroiditis and panuveitis