Abstract:The study describes 62 patients seen over a period of 4 years mainly presenting with an acute inflammatory ankle arthritis/periarthritis associated with panniculitis. Based upon the clinical characteristics of panniculitis two distinct categories could be defined. These included patients with characteristic lesions of erythema nodosum, seen in 51 (82%), the idiopathic form in 29 (57%), and secondary EN in 22 (43%). Associated conditions included Behçet's disease, oral contraceptive pills, pregnancy and penicil… Show more
“…It is suggested that T-cell immune response is involved in the pathogenesis of WCD. [2][3][4] In our patient, histochemistry staining showed lymphocytic cells in endobronchitis. Comparing tocyclosporine A, cyclophosphamide can decrease both T cell and B cell functions and replication.…”
Section: Discussionsupporting
confidence: 51%
“…Previous case reports showed cyclosporine A can be effective to resolve fever and painful subcutaneous nodules, and it can decrease high serum concentrations of interferon‐gamma and soluble interleukin‐2 in WCD patients. It is suggested that T‐cell immune response is involved in the pathogenesis of WCD . In our patient, histochemistry staining showed lymphocytic cells in endobronchitis.…”
Section: Discussionmentioning
confidence: 48%
“…1,2 It is also known as idiopathic relapsing febrile lobular nonsuppurative panniculitis and characterized by recurrent subcutaneous inflammatory painful nodules, fever and malaise due to systemic inflammation. [2][3][4] In severe cases, the inflammation can involve the lung, heart, gastrointestinal tract, spleen, kidney and adrenal gland. However, the prognosis of WCD is extremely variable.…”
Weber-Christian disease (WCD) is a chronic recurrent organ disease characterized by the formation of painful dense nodes in subcutaneous fat tissues, free from suppuration and accompanied by episodic temperature changes, chills and muscular pain. Sometimes it can involve the visceral organs. WCD often relapses during irregular intervals. It is a rare autoimmune inflammatory disorder of subcutaneous adipose tissue. However, lung involvement in WCD is rare. Here we report a man presenting with lung nodules. He was diagnosed as having WCD and successfully treated by corticosteroid and immonosuppressive agents. A 53-year-old male presented with a 2.5-year history of painful subcutaneous nodules on his trunk and limbs, accompanied by fever, night sweats, fatigue and malaise. A skin biopsy showed fat degeneration with foamy cells and infiltration of mononuclear cells. He was diagnosed as panniculitis and effectively treated by corticosteroid therapy at first. However, symptoms relapsed when the dosage of corticosteroid was tapered, and multiple lung nodules were found with the symptom of dyspnea in chest computed tomography scan during the follow-up period. Histological examination of the biopsy specimens from the lung was compatible with panniculitis change in the subcutanous nodule. Combination therapy of corticosteroid with cyclophosphamide was effective during the following 27 months.
“…It is suggested that T-cell immune response is involved in the pathogenesis of WCD. [2][3][4] In our patient, histochemistry staining showed lymphocytic cells in endobronchitis. Comparing tocyclosporine A, cyclophosphamide can decrease both T cell and B cell functions and replication.…”
Section: Discussionsupporting
confidence: 51%
“…Previous case reports showed cyclosporine A can be effective to resolve fever and painful subcutaneous nodules, and it can decrease high serum concentrations of interferon‐gamma and soluble interleukin‐2 in WCD patients. It is suggested that T‐cell immune response is involved in the pathogenesis of WCD . In our patient, histochemistry staining showed lymphocytic cells in endobronchitis.…”
Section: Discussionmentioning
confidence: 48%
“…1,2 It is also known as idiopathic relapsing febrile lobular nonsuppurative panniculitis and characterized by recurrent subcutaneous inflammatory painful nodules, fever and malaise due to systemic inflammation. [2][3][4] In severe cases, the inflammation can involve the lung, heart, gastrointestinal tract, spleen, kidney and adrenal gland. However, the prognosis of WCD is extremely variable.…”
Weber-Christian disease (WCD) is a chronic recurrent organ disease characterized by the formation of painful dense nodes in subcutaneous fat tissues, free from suppuration and accompanied by episodic temperature changes, chills and muscular pain. Sometimes it can involve the visceral organs. WCD often relapses during irregular intervals. It is a rare autoimmune inflammatory disorder of subcutaneous adipose tissue. However, lung involvement in WCD is rare. Here we report a man presenting with lung nodules. He was diagnosed as having WCD and successfully treated by corticosteroid and immonosuppressive agents. A 53-year-old male presented with a 2.5-year history of painful subcutaneous nodules on his trunk and limbs, accompanied by fever, night sweats, fatigue and malaise. A skin biopsy showed fat degeneration with foamy cells and infiltration of mononuclear cells. He was diagnosed as panniculitis and effectively treated by corticosteroid therapy at first. However, symptoms relapsed when the dosage of corticosteroid was tapered, and multiple lung nodules were found with the symptom of dyspnea in chest computed tomography scan during the follow-up period. Histological examination of the biopsy specimens from the lung was compatible with panniculitis change in the subcutanous nodule. Combination therapy of corticosteroid with cyclophosphamide was effective during the following 27 months.
“…Weber–Christian disease is the most serious form of panniculitis, with systemic manifestations, and has been only exceptionally described in the pediatric age group 1,24–28 . Seventy‐five percent of the cases occur in women after the second decade of life 26,29 .…”
EN is the most frequent panniculitis, with a benign course and is mainly associated with infections. WCD is a severe disease, with systemic involvement, that proceeds with cutaneous atrophy and requires the use of corticosteroids and or immunosuppressive drugs.
“…Lipophagia and granulomas led to confusion in the context of erythema nodosum, but focal necrosis of fat cells is not typical for it [3]. Erythema nodosum has been classi ed as predominantly septal panniculitis, unlike ndings in PWCD being classi ed as lobular panniculitis [10,11].…”
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.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.