Literature DB >> 3887095

Weber-Christian disease. Analysis of 15 cases and review of the literature.

R S Panush, R A Yonker, A Dlesk, S Longley, J R Caldwell.   

Abstract

We report 15 patients encountered over 13 years who presented with inflammation of subcutaneous fat and were given clinical and pathologic diagnoses of Weber--Christian disease (WCD). Prominent clinical features included female predominance, lower extremity nodules, fevers, arthritis/arthralgias, and myalgias. Notable laboratory features were elevated erythrocyte sedimentation rate, anemia, leukopenia, and hypocomplementemia, frequently with circulating 7S IgM or immune complexes at times of active symptoms. Histologic findings were lobular--together with frequent septal--panniculitis, fat-laden macrophages, variable cellular infiltrates, necrosis, and occasional vasculitis. Follow-up revealed the death of 2 patients and disease stabilization or improvement in 13 patients. Six patients developed features of other diseases (factitial disease, erythema nodosum, acute myelogenous leukemia, rheumatoid arthritis, systemic lupus erythematosus, and sarcoid) and a seventh may have had erythema induratum. We suggest that classic WCD, as originally described, reflects an increasingly recognized spectrum of panniculitides. These are syndromes of diverse etiology that share many clinical, inflammatory, and immunologic features.

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Year:  1985        PMID: 3887095

Source DB:  PubMed          Journal:  Medicine (Baltimore)        ISSN: 0025-7974            Impact factor:   1.889


  10 in total

1.  Weber-Christian disease producing splenic vein occlusion and bleeding gastric varices: successful treatment with sclerotherapy.

Authors:  D Heseltine; M Bramble; A Cole; D Clarke; W Castle
Journal:  Postgrad Med J       Date:  1990-04       Impact factor: 2.401

2.  Weber-Christian disease with calcinosis: a case report.

Authors:  D Biasi; P Caramaschi; A Carletto; S Zeminian; L M Bambara
Journal:  Clin Rheumatol       Date:  1996-11       Impact factor: 2.980

3.  Visceral brown fat necrosis in postperinatal mortality.

Authors:  T J Stephenson; S Variend
Journal:  J Clin Pathol       Date:  1987-08       Impact factor: 3.411

4.  Systemic Weber-Christian disease with reversible bilateral ureteric obstruction.

Authors:  L A Brawn; L E Ramsay; J R Shortland; J L Williams
Journal:  Postgrad Med J       Date:  1989-06       Impact factor: 2.401

5.  Weber-Christian disease with ileocolonic involvement successfully treated with infliximab.

Authors:  José Miranda-Bautista; Alejandro Fernández-Simón; Isabel Pérez-Sánchez; Luis Menchén
Journal:  World J Gastroenterol       Date:  2015-05-07       Impact factor: 5.742

6.  Efficacy of cyclosporine A treatment in relapsing febrile lobular panniculitis associated with small vessel vasculitis.

Authors:  Luca Cantarini; Francesca Fanti; Mauro Galeazzi; Silvana Martino; Clelia Miracco; Maurizio Biagioli; Norberto Dal Canto; Rolando Cimaz
Journal:  Rheumatol Int       Date:  2009-06-09       Impact factor: 2.631

7.  Weber-Christian panniculitis with systemic cytophagic histiocytosis.

Authors:  H Steininger; M Missmahl
Journal:  Klin Wochenschr       Date:  1988-04-15

8.  Weber-Christian disease presenting with intractable fever and periorbital swelling mimicking angioedema.

Authors:  H P Wang; C C Huang; C H Chen; H Y Lin
Journal:  Clin Rheumatol       Date:  2006-03-21       Impact factor: 3.650

9.  Idiopathic pleural panniculitis with recurrent pleural effusion not associated with Weber-Christian disease.

Authors:  Paolo Laperuta; Filomena Napolitano; Rosa Maria Di Crescenzo; Pio Zeppa; Antonio Galderisi; Carmine Selleri; Alessandro Vatrella; Mario Capunzo; Vincenzo Giuseppe Di Crescenzo
Journal:  Open Med (Wars)       Date:  2016-11-19

10.  Pfeifer-Weber-Christian Disease during Pregnancy Successfully Treated with Corticosteroids.

Authors:  Eftychia Platsidaki; Ourania Kotsafti; Anargyros Kouris; Efthymia Agiasofitou; Dorothea Polydorou
Journal:  Indian J Dermatol       Date:  2016 Sep-Oct       Impact factor: 1.494

  10 in total

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