syndrome is a newly described autoinflammatory, genetic disorder.
The acronym CANDLE syndrome was first introduced by Torrelo et al in 2010.
4 patients had unique clinical, histopathological, and laboratory features.
CAND = Chronic Atypical Neutrophilic Dermatosis
L = Lipodystrophy
E = Elevated temperature
The patients present with early onset recurrent fever.
The skin lesions are characterized by annular violaceous plaques.
Histopathologically, skin biopsy shows inflammatory reaction consisting of both mature neutrophils and some mononuclear cells without vasculitis.
Perivascular and interstitial infiltrates of atypical mononuclear cells of myeloid lineage are noted.
There is persistent eyelid and lip swelling, delayed physical development, progressive lipodystrophy, hepatomegaly, and a range of visceral inflammatory manifestations.
Most of the patients have been shown to have mutation in PSMB8 gene.
Pathology Acronyms are a fun way to remember long, difficult and rare names of diseases. In an article Kerl et al have used CAMEL, METRO to remember two rare collision tumours - METRO (MElanocytic tumor +TRichOblastoma) and CAMEL (CArcinoma +MELanoma).
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