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.


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.


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.


Autoinflammatory Alliance

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


Homepage | FAQs | Contact Dr. White

It is not the intention of to provide specific medical advice, diagnosis or treatment. only intends to provide users with information regarding various medical conditions for educational purposes and will not provide specific medical advice. Information on is not intended as a substitute for seeking medical treatment and you should always seek the advice of a qualified healthcare provider for diagnosis and for answers to your individual questions. Information contained on should never cause you to disregard professional medical advice or delay seeking treatment. If you live in the United States and believe you are having a medical emergency call 911 immediately.