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Neutrophilic Lobular Panniculitis as an Expression of a Widened Spectrum of Familial Mediterranean Fever

María Leiva-Salinas, MD1; Isabel Betlloch, MD, PhD1; María Paloma Arribas, MD1; Laura Francés, MD1; Jose Carlos Pascual, MD1
[+] Author Affiliations
1Department of Dermatology, Hospital General Universitario de Alicante, Alicante, Spain
JAMA Dermatol. 2014;150(2):213-214. doi:10.1001/jamadermatol.2013.6095.
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Familial Mediterranean fever (FMF) is considered to be an autosomal recessive disease, though it is controversial.13 The marenostrin-encoding fever gene (MEFV) is responsible for FMF. The most frequent mutation is M694V, which represents a genetic risk factor for development of amyloidosis1 and more severe disease. The classic clinical picture consists of generally short recurrent febrile episodes, serositis, and erysipelas-like erythema (ELE). Colchicine is the treatment of choice for prevention of the attacks and AA amyloidosis. With isolation of the MEFV gene in 1997, incomplete or “atypical” forms of FMF have been reported and are considered by some authors an expanded spectrum of the disease.2

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Figures

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Figure 1.
Recurrent Contusiform Nodules on the Lower Limbs

Tender, erythematous, contusiform, warm nodules with irregular shape, located on the lower limbs.

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Figure 2.
Lobular Neutrophilic Panniculitis

Punch biopsy specimens were taken and stained with hematoxylin-eosin. A, Predominantly lobular neutrophilic panniculitis without evidence of necrosis, vasculitis, crystals, or destruction of adipocytes (original magnification ×20). B, Lipophagic histiocytes and giant cells (original magnification ×40).

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