Case & reviewChronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome
Section snippets
Patient 1
A boy with an unremarkable family history was first seen at 8 years of age because of fever and skin lesions, both of which appeared during the first month of life. Fever higher than 38.5 °C had been present almost daily, mainly in the morning, and remitted only partially with administration of nonsteroidal anti-inflammatory drugs (NSAIDs). Skin lesions consisted of 1- to 5-cm annular erythematous, edematous plaques, mainly affecting the face and trunk that became purpuric and cleared after
Discussion
These 4 patients had similar cutaneous and systemic findings (Table I). The characteristic violaceous plaques were distinctive clinically and histopathologically. Features common to all patients were early disease onset, daily or almost daily fever with a poor response to administration of NSAIDs, delayed physical development, recurrent purpuric skin lesions, typical facial features with violaceous swollen eyelids and thick lips, progressive lipodystrophy, and arthralgia without arthritis.
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Conflicts of interest: None declared.