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Correspondence |

Late-Onset Familial Mediterranean Fever: An Atypical Presentation of Dermatologic Interest

Rosanna Satta, MD; Laura Obici, MD; Giampaolo Merlini, MD; Francesca Cottoni, MD
Arch Dermatol. 2007;143(8):1073-1087. doi:10.1001/archderm.143.8.1080.
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Familial Mediterranean fever (FMF) is a recessively inherited autoinflammatory disease characterized by self-limited bouts of fever and polyserositis. Onset usually occurs in childhood, and reactive amyloidosis may be a life-threatening complication in adult life.1 As for other autoinflammatory syndromes, the skin is an important target tissue for inflammation. In FMF, the appearance of an erysipeloid erythema, typically monolateral and localized to the lower leg and on the dorsal surface of the foot,2 is pathognomonic in the context of systemic inflammation, including fever, serositis, and synovitis. However, the clinical significance of such a cutaneous feature is difficult to define in patients without a disease-associated history or in the absence of a positive genetic test finding.

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Figure 1.

Recurrent edematous, erythematous erysipeloid patch on the left thigh.

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Figure 2.

A skin biopsy specimen revealed edema of the derma with interstitial and perivascular neutrophil and lymphocytic infiltrate and thrombosis of a few small vessels without vasculitis (hematoxylin-eosin). A, Edema in the superficial derma and interstitial and perivascular inflammatory infiltrate (original magnification ×20). B, Higher-magnification image of perivascular neutrophilic and lymphocytic infiltrate (original magnification ×40).

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