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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
Summary
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.
- 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.