Weber-Christian disease presenting with lung nodules dramatically improved with corticosteroid therapy: one case report and literature review

Int J Rheum Dis. 2018 Feb;21(2):573-578. doi: 10.1111/1756-185X.12620. Epub 2015 Jul 29.

Abstract

Weber-Christian disease (WCD) is a chronic recurrent organ disease characterized by the formation of painful dense nodes in subcutaneous fat tissues, free from suppuration and accompanied by episodic temperature changes, chills and muscular pain. Sometimes it can involve the visceral organs. WCD often relapses during irregular intervals. It is a rare autoimmune inflammatory disorder of subcutaneous adipose tissue. However, lung involvement in WCD is rare. Here we report a man presenting with lung nodules. He was diagnosed as having WCD and successfully treated by corticosteroid and immonosuppressive agents. A 53-year-old male presented with a 2.5-year history of painful subcutaneous nodules on his trunk and limbs, accompanied by fever, night sweats, fatigue and malaise. A skin biopsy showed fat degeneration with foamy cells and infiltration of mononuclear cells. He was diagnosed as panniculitis and effectively treated by corticosteroid therapy at first. However, symptoms relapsed when the dosage of corticosteroid was tapered, and multiple lung nodules were found with the symptom of dyspnea in chest computed tomography scan during the follow-up period. Histological examination of the biopsy specimens from the lung was compatible with panniculitis change in the subcutanous nodule. Combination therapy of corticosteroid with cyclophosphamide was effective during the following 27 months.

Keywords: Weber-Christian disease; lung nodules; panniculitis; response.

Publication types

  • Case Reports
  • Letter
  • Review

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use*
  • Biopsy
  • Cyclophosphamide / therapeutic use
  • Drug Therapy, Combination
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Male
  • Methylprednisolone / therapeutic use*
  • Middle Aged
  • Multiple Pulmonary Nodules / diagnosis
  • Multiple Pulmonary Nodules / drug therapy*
  • Multiple Pulmonary Nodules / etiology
  • Panniculitis, Nodular Nonsuppurative / complications
  • Panniculitis, Nodular Nonsuppurative / diagnosis
  • Panniculitis, Nodular Nonsuppurative / drug therapy*
  • Positron Emission Tomography Computed Tomography
  • Treatment Outcome

Substances

  • Adrenal Cortex Hormones
  • Immunosuppressive Agents
  • Cyclophosphamide
  • Methylprednisolone