title: Familial Mediterranean fever variant with repeated atypical skin eruptions.
abstract: Familial Mediterranean fever (FMF) is characterized by self-limited bouts of fever and polyserositis. Skin involvement is not common in FMF, and erysipelas-like erythema is found to be the most frequent skin eruption which is often accompanied by arthritis and fever, and disappears within 12-72 h. We report a 40-year-old Japanese woman who presented with a 2-year history of recurrent fever with general fatigue, polyarthralgia and transient maculopapular eruptions on her lower extremities and trunk. The histological findings of the maculopapular eruption showed lymphocyte infiltration around the capillaries in the entire dermis. Mutation analysis showed a heterozygous E148Q-P369S mutation of MEFV. These findings suggested a diagnosis of late-onset FMF variant with atypical skin eruptions. The patient was successfully treated with colchicine. Thus, we should pay attention to repeated atypical skin eruptions for the early detection of atypical FMF.
authors.affiliation: Department of Dermatology, Gifu University Graduate School of Medicine, Gifu, Japan.
authors.forename: Tomoko
authors.initials: T
authors.lastname: Takahashi
authors.affiliation: Department of Dermatology, Gifu University Graduate School of Medicine, Gifu, Japan.
authors.forename: Tomomi
authors.initials: T
authors.lastname: Fujisawa
authors.affiliation: Department of General and Cardiothoracic Surgery, Gifu University Graduate School of Medicine, Gifu, Japan.
authors.forename: Masaki
authors.initials: M
authors.lastname: Kimura
authors.affiliation: Department of Pediatrics, Gifu University Graduate School of Medicine, Gifu, Japan.
authors.forename: Hidenori
authors.initials: H
authors.lastname: Ohnishi
authors.affiliation: Department of Dermatology, Gifu University Graduate School of Medicine, Gifu, Japan.
authors.forename: Mariko
authors.initials: M
authors.lastname: Seishima
mesh_terms: D000328:Adult; D003078:Colchicine / Q000627:therapeutic use; D010505:Familial Mediterranean Fever / Q000188:drug therapy / Q000473:pathology*; D005260:Female; D006801:Humans; D012867:Skin / Q000473:pathology*; D050257:Tubulin Modulators / Q000627:therapeutic use
chemical_list: D050257:Tubulin Modulators; D003078:Colchicine
keywords: MEFV; autoinflammatory disease; colchicine; erysipelas-like erythema; familial Mediterranean fever