title: Recurrent migratory angioedema as cutaneous manifestation in a familiar case of TRAPS: dramatic response to Anakinra.
abstract: BACKGROUND
  Tumor Necrosis Factor Receptor Associated Periodic Syndrome (TRAPS) is a hereditary autoinflammatory syndrome characterized by recurrent episodes of fever and localized inflammation. Clinical presentation can be very variable in terms of duration of fever attacks, periodicity, and accompanying manifestations. One of the most characteristic symptoms is the occurrence of migrating skin rash with myalgia that is sustained by monocytic inflammation.
  
  
  METHODS
  We herein present the case of a family suffering from TRAPS who had been misdiagnosed for a long period of time and whose main symptom was migrating angioedema. Skin biopsy from one of the patients documented a monocytic panniculitis. All the living patients responded dramatically to anakinra treatment.
  
  
  CONCLUSIONS
  The classic symptom of migratory angioedema with myalgia in TRAPS can be produced by monocytic panniculitis.This manifestation is so characteristic of TRAPS that its occurrence, even in the absence of other manifestations, should prompt genetic analysis. Our patient's condition responded promptly to anakinra treatment.
authors: University of Brescia.
authors: M
authors: M
authors: Cattalini
authors: A
authors: A
authors: Meini
authors: P
authors: P
authors: Monari
authors: G
authors: G
authors: Gualdi
authors: M
authors: M
authors: Arisi
authors: F
authors: F
authors: Pelucchi
authors: S
authors: S
authors: Bolognini
authors: M
authors: M
authors: Gattorno
authors: P G
authors: PG
authors: Calzavara-Pinton
authors: A
authors: A
authors: Plebani
mesh_terms: D000799:Angioedema / Q000209:etiology*; D018501:Antirheumatic Agents / Q000627:therapeutic use*; D005260:Female; D005334:Fever; D056660:Hereditary Autoinflammatory Diseases / Q000175:diagnosis / Q000188:drug therapy* / Q000235:genetics; D006801:Humans; D053590:Interleukin 1 Receptor Antagonist Protein / Q000627:therapeutic use*; D008297:Male; D009154:Mutation; D047888:Receptors, Tumor Necrosis Factor, Type I / Q000235:genetics; D012008:Recurrence; D055815:Young Adult
chemical_list: D018501:Antirheumatic Agents; D053590:Interleukin 1 Receptor Antagonist Protein; D047888:Receptors, Tumor Necrosis Factor, Type I