By Gary M. White, MD

Chronic Atypical Neutrophilic Dermatosis with Lipodystrophy and Elevated temperature (CANDLE) syndrome is a new auto-inflammatory syndrome caused by mutations in the gene for proteasome subunit, beta type, 8 (PSMB8).


The typical patient is an infant with fever and recurrent annular erythematous and violaceous, plaques that may leave purpura upon healing. Later during infancy, the eyelids become swollen and erythematous or violaceous. The lips may be thick and there may be a progressive lipodystrophy. Non-cutaneous findings include fevers, delayed physical development, microcytic anaemia and arthralgia.


Treatment usually involves high dose corticosteroids, e.g., prednisone 1-2 mg/kg/day which improves the skin lesions, joint pain and fever. The JK inhibitor baricitinib is being investigated. w


British J Dermatology 2014 January

JAAD March 2010 Volume 62, Issue 3, Pages 489–495

Biomed Res Int. 2014; 2014: 616973.

Example Case

A 3 month-old infant presents with fever and recurrent annular erythematous and violaceous plaques that leave purpura. The rash resembles urticarial vasculitis. Later in infancy the eyelids become swollen and erythematous. As the child grows, the lips become thick and the cheeks sunken (lipodystrophy).


Homepage | FAQs | Use of Images | 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.