Defects in CIAS1 are a cause of familial cold autoinflammatory syndrome. FCAS is rare autosomal dominant systemic inflammatory disease characterized by episodes of rash, arthralgia, fever and conjunctivitis after generalized exposure to cold.
Alternative names
FCAS
Familial cold autoinflammatory syndrome, Cold hypersensitivity, Familial cold-induced autoinflammatory syndrome, Familial polymorphous cold eruption
Classification
- Periodic fever syndromes
- Cold autoinflammatory syndrome
Inheritance
Autosomal dominant
Cross references
Phenotypically related immunodeficiencies
IDR factfile for Chronic infantile neurological cutaneous and articular syndrome
IDR factfile for Granulomatous sinovitis with uveitis and cranial neuropathies
Incidence
Incidence unknown.