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Nodular non-suppurative panniculitis

Orpha number ORPHA33577
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
ICD-O -
OMIM -
UMLS
  • C0030328
MeSH
  • D010201
MedDRA
  • 10047883

Summary

Nodular non-suppurative panniculitis, known as Weber-Christian disease (WCD), is a rare skin disorder characterized by recurring inflammation in the subcutaneous layer of fat. The exact prevalence is unknown. It occurs more often in adult women (approximately 75% of reported cases). 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 remains unknown. 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 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). 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.

Expert reviewer(s)

  • Dr Giovanna ZAMBRUNO

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