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Nodular non-suppurative panniculitis
Disease definition
A rare skin disorder characterized by recurring inflammation in the subcutaneous layer of fat.
ORPHA:33577
Classification level: Disorder- Synonym(s):
- Idiopathic lobular panniculitis
- Idiopathic nodular panniculitis
- Pfeiffer-Weber-Christian syndrome
- Relapsing febrile nodular nonsuppurative panniculitis
- Relapsing febrile nodular panniculitis
- WCD
- Weber-Christian disease
- Weber-Christian panniculitis
- Prevalence: Unknown
- Inheritance: Not applicable
- Age of onset: Adult
- ICD-10: M35.6
- OMIM: -
- UMLS: C0030328
- MeSH: D010201
- GARD: 7879
- MedDRA: 10047883
Summary
Epidemiology
Nodular non-suppurative panniculitis, known as Weber-Christian disease (WCD) prevalence is unknown. It occurs more often in adult women (approximately 75% of reported cases).
Clinical description
WCD is characterized by single or multiple, tender or painful edematous and often erythematous subcutaneous nodules (1-2 cm large, most often affecting the lower extremities) healing with a depressed scar. The upper extremities, buttocks, abdominal wall, breasts, and face can also be involved. In most cases WCD is associated with fever, malaise, myalgia, arthralgia, and abdominal pain. Nausea, vomiting, weight loss, and hepatomegaly may occur. The disease tends to recur at intervals of weeks or months. WCD can present as a severe systemic illness. Unusual nodule locations include the abdomen (as an abscess), mesentery of the small or large bowel (with bowel obstruction), lungs, heart, liver, spleen, adrenal glands, kidneys, scrotum, and cranium. In patients with systemic disease, morbidity and mortality are significant.
Etiology
Etiology remains unknown.
Diagnostic methods
Histological examination of a subcutaneous nodule is necessary to confirm the diagnosis (the fat lobules are replaced by neutrophils, lymphocytes, and histiocytes in the early stages, and by macrophages and fibrotic tissue later).
Differential diagnosis
Differential diagnosis includes erythema nodosum and erythema induratum of Bazin, which represent the commonest forms of predominantly septal panniculitis and predominantly lobular panniculitis with vasculitis, respectively, as well as a variety of distinctive disease entities associated with panniculitis. The latter comprise bacterial, viral and fungal infections, connective tissue disorders, disturbances of lipid metabolism, pancreatic and renal diseases, gout, lymphoproliferative neoplasia, trauma, alpha1-antitrypsin deficiency (see this term).
Management and treatment
Treatment is symptomatic and supportive and may include systemic steroids and nonsteroidal anti-inflammatory drugs. Responses to fibrinolytic agents, hydroxychloroquine, azathioprine, thalidomide, cyclophosphamide, tetracycline, and cyclosporin A have been reported in some patients.
Additional information