MUCKLE-WELLS SYNDROME

By Gary M. White, MD


The Muckle-Wells Syndrome is a rare autosomal dominant disease characterized by recurrent episodes of fever, urticaria, arthralgia, myalgia and conjunctivitis from childhood.

Clinical

The main features of this disease are an urticaria-like eruption that begins in infancy, progressive perceptive deafness, and amyloid nephropathy. The urticarial lesions are often accompanied by fever, arthralgias and limb pain.   A characteristic erysipelas rash may occur about the ankles.

Treatment

Cankinumab (ILARIS) is an interleukin-1β blocker indicated for the treatment of cryopyrin-associated periodic syndromes (CAPS) in adults and children 4 years of age and older including: familial cold autoinflammatory syndrome (FCAS).

Anakinra is a recombinant, nonglycosylated form of the human interleukin-1 receptor antagonist. In one case report [Clin and Exp Derm 2013;38;875], a patient experienced complete resolution of symptoms with anakinra 100 mg SQ initially, tapered to 50 mg/day.

References

Autoinflammatory Alliance

Urticarial rash every afternoon and erysipelas-like rash in cryopyrin-associated periodic syndromes (CAPS). Arthritis Res Ther. 2009; 11(1): 212

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