Recurrent panniculitis: Weber-Christian disease

Hagag, Adel A; Barakat, Amany Nagy

doi:10.1177/1721727x16659072

Cited by 3 publications

(5 citation statements)

References 14 publications

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“…They are derived on the basis of individual cases. Drugs used in the treatment of PWCD include corticosteroid therapy, brinolytic agens, hydroxychloroquine, azathioprine, thalidomide, cyclophosphamide, tetracycline, CyA, mycophenolate mofetil and anti-TNF treatment [4,[16][17][18][19]. CyA and corticosteroids have been proved most effective [4,[20][21][22].…”

Section: Case Presentationmentioning

confidence: 99%

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Pfeifer-Weber-Christian Disease and Benign Multiple Subcutaneous Noninfiltrative Angiolipomas: A Puzzling Case and Review of a Rare Entitety

Radunović¹,

Pavlov-Dolijanovic²,

Miličić³

et al. 2020

Preprint

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Background: Pfeifer-Weber-Christian disease (PWCD) is one of many rare diseases that may be easily missed if there is not a high degree of suspicion. Angiolipomas are rare, benign subcutaneous tumors, composed of adipose tissue and blood vessels and often containing fibrin thrombi. The majority of angiolipomas occur sporadically; however, there is a minority of cases that have been associated with long-term corticosteroid use. Case presentation: We report here an unusual case of PWCD associated with benign multiple subcutaneous noninfiltrative angiolipomas confirmed by skin biopsy. Systemic corticosteroid therapy was not effective at reducing flares of panniculitis, and during this therapy angiolipomas gradually increased in size. In contrast, administration of oral Cyclosporine A (CyA) led to a rapid remission of the PWCD. Conclusions: As PWCD has no known aetiology and no specific treatment has been established, the successful therapy with the CyA supports the hypothesis that PWCD is a T cell mediated autoinflammatory condition. Also, this case represent very rare side effects of corticosteroid therapy, such as induction of de novo angiolipomas or increased growth of existing tumours. This case bring diagnostic difficulties in everyday clinical practice, especially in patients with panniculitides, but histopathological evaluation usually resolves the dilemma.

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Section: Case Presentationmentioning

confidence: 99%

“…Etiopathogenesis of the disease is not fully known, participation of autoimmune mechanisms is anticipated. The diagnosis of PWCD is a diagnosis of exclusion when no other cause for the lobular panniculitis can be identi ed [3,4].…”

Section: Introductionmentioning

confidence: 99%

Pfeifer-Weber-Christian Disease and Benign Multiple Subcutaneous Noninfiltrative Angiolipomas: A Puzzling Case and Review of a Rare Entitety

Radunović¹,

Pavlov-Dolijanovic²,

Miličić³

et al. 2020

Preprint

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“…44 Cyclosporine A (CsA) is able to block the transcription of cytokine genes in activated T cells. Hagag and Barakat 24 reported a good efficacy of CsA (5 mg/kg/die for 6 months) in a child with hematological and hepatosplenomegaly. CsA (5 mg/Kg/day) had been used with success in an 8-year-old boy with skin lesion and small vessels vasculitis.…”

Section: Treatment Choicesmentioning

confidence: 99%

“…The clinical features of hematological involvement are anemia 1,5,19,23,32 (mostly hypochromic and microcytic, 3,14,24 occasionally normochromic and microcytic) 16 , leucopenia 3,6,20 (with mainly neutropenia), 19 and thrombocytopenia. 5,11 Pancytopenia is rarely described.…”

Section: Hematological Manifestationmentioning

confidence: 99%

“…6,20 However, it is described that AZA failed to reach complete remission in 2 cases. 24,37 Cyclophosphamide (CyC), a synthetic alkylating agent, which metabolite forms irreversible DNA interstrand and intrastrand crosslinks, leading to cell apoptosis. 21 Kirch et al 21 used CyC (200 mg/daily) in a patient with hematological and liver involvement and glomerulonephritis, describing a treatment discontinuation due to the occurrence of leucopenia and thrombocytopenia.…”

Section: Treatment Choicesmentioning

confidence: 99%

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Pfeifer-Weber-Christian Disease: A Case Report and Review of Literature on Visceral Involvements and Treatment Choices

Rotondo

Corrado

Mansueto

et al. 2020

Clin Med Insights Case Rep

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Pfeifer-Weber-Christian disease (PWCD) is a rare idiopathic disease characterized by lobular panniculitis of adipose tissue with systemic symptoms and multiple organ involvement. Even though the systemic involvement is rare, it is life-threatening and represent a treatment challenge for the clinicians. We report a case of PWCD characterized by hepatic, hematologic, and renal involvement, with good response to mofetil mycophenolate and prednisone treatment. A 47-year-old female presented several months’ history of painful subcutaneous nodules, fever and lymphadenopathy with recent appearing of microcytic hypochromic anemia, leucopenia with neutropenia, and increase in transaminase. Skin biopsy showed lobular panniculitis with lymph-histiocytic and neutrophilic infiltrates with necrosis of adipocytes. A combination therapy of corticosteroid with mofetil mycophenolate was effective. Moreover, we discuss the clinical manifestation and the therapeutic choices in PWCD, from classical immunosuppressive drugs to new biotechnological agents, and we provide a comprehensive review of the available literature.

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Eritema nudoso crónico de localización generalizada: a considerar enfermedad de Weber Christian

Briceño-Moya

Hurtado-Díaz

Espinoza-Sánchez

2021

RMIMSS

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Recurrent panniculitis: Weber-Christian disease

Cited by 3 publications

References 14 publications

Pfeifer-Weber-Christian Disease and Benign Multiple Subcutaneous Noninfiltrative Angiolipomas: A Puzzling Case and Review of a Rare Entitety

Pfeifer-Weber-Christian Disease and Benign Multiple Subcutaneous Noninfiltrative Angiolipomas: A Puzzling Case and Review of a Rare Entitety

Pfeifer-Weber-Christian Disease: A Case Report and Review of Literature on Visceral Involvements and Treatment Choices

Eritema nudoso crónico de localización generalizada: a considerar enfermedad de Weber Christian

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