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Relapsing polychondritis and Weber-Christian disease.

C R Smith1, E H Sawicka, E Sheffield

  • 1Department of Rheumatology, King's College Hospital, London.

British Journal of Rheumatology
|December 1, 1988
PubMed
Summary
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A patient with relapsing polychondritis experienced significant abdominal pain, which led to the diagnosis of Weber-Christian disease (systemic panniculitis) through biopsies. This case highlights an unusual association with important prognostic and therapeutic considerations.

Area of Science:

  • Rheumatology
  • Dermatology
  • Gastroenterology

Background:

  • Relapsing polychondritis (RP) is a rare autoimmune disease affecting cartilage.
  • Weber-Christian disease (WCD), or systemic panniculitis, involves inflammation of subcutaneous fat.
  • The co-occurrence of RP and WCD is exceptionally rare in medical literature.

Observation:

  • A patient presented with classical relapsing polychondritis.
  • Abdominal pain was a primary and prominent symptom in this patient.
  • Clinical suspicion for Weber-Christian disease was raised due to the presentation.

Findings:

  • Histological examination of skin biopsies confirmed systemic panniculitis (Weber-Christian disease).
  • Mesenteric biopsies also supported the diagnosis of Weber-Christian disease.

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  • The findings confirmed an unusual association between relapsing polychondritis and Weber-Christian disease.

    • Implications:

    • This case underscores the importance of considering systemic panniculitis in patients with relapsing polychondritis presenting with abdominal pain.
    • Understanding this rare disease association is crucial for accurate prognostication.
    • Therapeutic strategies may need to be tailored to address both conditions simultaneously.