Case & review
Chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (CANDLE) syndrome

https://doi.org/10.1016/j.jaad.2009.04.046Get rights and content

Several syndromes manifest as recurrent daily fevers, skin lesions, and multisystem inflammation. We describe 4 patients with early-onset recurrent fevers, annular violaceous plaques, persistent violaceous eyelid swelling, low weight and height, lipodystrophy, hepatomegaly, and a range of visceral inflammatory manifestations. Laboratory abnormalities included chronic anemia, elevated acute-phase reactants, and raised liver enzymes. Histopathologic examination of lesional skin showed atypical mononuclear infiltrates of myeloid lineage and mature neutrophils. Our patients have a distinctive early-onset, chronic inflammatory condition with atypical or immature myeloid infiltrates in the skin. We propose the acronym CANDLE (chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature) syndrome for this newly described disorder, which is probably genetic in origin.

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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    Funding sources: None.

    Conflicts of interest: None declared.

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