Familial cold urticaria and Muckle-Wells syndrome

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

OMIM

#120100 Familial cold autoinflammatory syndrome

#191900 Muckle-Wells syndrome

*606416 CIAS1 gene; CIAS1

Incidence

Incidence unknown.