Schnitzler's syndrome: a diagnostic conundrum

BMJ Case Rep. 2014 Jun 25:2014:bcr2013202210. doi: 10.1136/bcr-2013-202210.

Abstract

A 63-year-old man was referred to the rheumatology unit with myalgia, arthralgia and a widespread rash. He was investigated for rheumatological disease; however, this did not yield any specific findings. His symptoms were attributed to statin use and newly started antihypertensives which were promptly discontinued. There was some subsequent improvement in his symptoms so he was discharged. He re-presented 3 years later with pelvic bone pain which had responded to a course of oral steroids started by the general practitioner, who was treating a presumed diagnosis of polymyalgia rheumatica. However, MRI of the pelvis showed bone marrow oedema and the diagnosis was changed to mastocytosis. He did not tolerate or respond to initial treatment. It was not until a second opinion was sought from the dermatologists that the diagnosis of Schnitzler's syndrome was made and treatment with anakinra (an interleukin-1 receptor antagonist) was started with almost complete resolution of his symptoms.

Publication types

  • Case Reports

MeSH terms

  • Antirheumatic Agents / therapeutic use*
  • Arthralgia / diagnosis*
  • Arthralgia / drug therapy
  • Arthralgia / etiology
  • Humans
  • Interleukin 1 Receptor Antagonist Protein / therapeutic use*
  • Male
  • Middle Aged
  • Myalgia / diagnosis*
  • Myalgia / drug therapy
  • Myalgia / etiology
  • Schnitzler Syndrome / diagnosis*
  • Schnitzler Syndrome / drug therapy
  • Schnitzler Syndrome / pathology
  • Urticaria / diagnosis*
  • Urticaria / drug therapy
  • Urticaria / etiology

Substances

  • Antirheumatic Agents
  • Interleukin 1 Receptor Antagonist Protein