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Small vessel vasculitis and relapsing panniculitis in tumour necrosis factor receptor associated periodic syndrome (TRAPS)

¹ Department of Rheumatology, University Hospital of Schleswig-Holstein, Campus Luebeck, and Rheumaklinik Bad Bramstedt, Luebeck, Germany
² Second Medical Department, University Hospital of Schleswig-Holstein, Campus Kiel, Kiel, Germany
³ Institute of Immunology, University Hospital of Schleswig-Holstein, Campus Kiel, Kiel, Germany
⁴ Department of Dermatology, University Hospital of Schleswig-Holstein, Campus Kiel, Kiel, Germany

Correspondence to:
Correspondence to:
Dr P Lamprecht
Department of Rheumatology, University Hospital of Schleswig-Holstein, Campus Luebeck, and Rheumaklinik Bad Bramstedt, Ratzeburger Allee 160, 23538 Luebeck, Germany; lamprecht{at}rheuma-zentrum.de

ABSTRACT
Case reports: A 66 year old female patient had relapsing fever and non-suppurative panniculitis suggestive of enigmatic "Weber-Christian disease" (WCD). Antineutrophil cytoplasmic antibodies with specificity for human leucocyte elastase (HLE-ANCA) were detected. A biopsy showed small vessel vasculitis and panniculitis. A 53 year old man had recurrent episodes of abdominal pain, erythematous rash, and myalgia. Fever attacks had stopped a few years ago. A biopsy showed panniculitis and fasciitis. In both patients mutations (R92Q, T50M) of the tumour necrosis factor receptor super family (TNFRSF) 1A gene were disclosed. Mutations of the TNFRSF 1A gene are the cause of tumour necrosis factor receptor associated periodic syndrome (TRAPS). Both patients responded favourably to treatment with the human soluble p75 TNF receptor fusion protein etanercept (2x25 mg subcutaneously/week).

Discussion: Small vessel vasculitis and panniculitis have not been reported in TRAPS so far. The cases underline the importance of TNF regulation in inflammatory processes including vasculitis. Genetically determined causes of fever may account for some cases of WCD.

Abbreviations: FMF, familial Mediterranean fever; HLE-ANCA, antineutrophil cytoplasmic antibodies with specificity for human leucocyte elastase; PCR, polymerase chain reaction; TNF, tumour necrosis factor ; TNFRSF, tumour necrosis factor receptor super family; TRAPS, tumour necrosis factor receptor associated periodic syndrome; WCD, "Weber-Christian disease"

Keywords: etanercept; panniculitis; tumour necrosis factor ; vasculitis

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