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Background: Weber-Christian disease is a recurrent, non-suppurative, febrile nodular panniculitis, more frequent in young women. It is characterized by recurrent outbreaks of subcutaneous nodules, distributed symmetrically. It has an idiopathic origin, or it is secondary to pancreatic disease, physicochemical agents or alpha-1 antitrypsin deficiency. It can affect any tissue with body fat. Histologically is a lobular panniculitis without vasculitis., Case Report: 23-year-old woman. She was admitted with subcutaneous painful nodules in pelvic, gluteus and forearms, with erythematous and hyperpigmented plaques; ecchymosis in the right upper eyelid and bilateral hyposphagma; fever of 38 °C; diaphoresis and oral ulcers, not painful. The hemogram showed pancytopenia, elevation of acute phase reagents, amylase and normal lipase. Blood culture was negative. Antinuclear antibodies and complement were normal. Computed tomography of the abdomen showed disseminated hyperdensity in subcutaneous tissue. The biopsy showed lobular panniculitis without vasculitis compatible with WeberChristian panniculitis. The patient was treated with steroids and immunosuppressive therapy., Conclusions: Infectious etiology and other causes of erythematous nodules were ruled out through clinical evolution and complementary studies. The patient presented spontaneous involution in several weeks and sometimes the lesions evolved to sterile abscesses, in addition to systemic clinical manifestations. Corticosteroid therapy was deemed successful., (Copyright: © 2020 Revista Medica del Instituto Mexicano del Seguro Social.)

Inflammatory conditions manifest with a broad spectrum of signs and symptoms. Panniculitis is such a condition affecting the subcutaneous fat and presents as tender erythematous nodules. It is also associated with a systemic response and has been described in the literature as early as in 1892 by Pfeifer and in the 1920s by Weber and Christian. We present an unusual case of a Caucasian man with recurrent febrile illness, systemic inflammatory response and renal dysfunction requiring acute high dependency care. The authors successfully treated him with an antiproliferative agent, mycophenolate mofetil, which resulted in preventing him from having further episodes., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Weber-Christian disease (W-CD) is associated with relapsing nodular panniculitis and a variety of systemic findings. Renal parenchymal involvement has been rarely reported. The authors describe a case of nephrotic syndrome in an African-American man with a W-CD flare. The patient had chills and low-grade fever with painful lower extremity skin lesions. A renal biopsy demonstrated the tip variant of focal segmental glomerulosclerosis (FSGS). The kidney biopsy also suggested parenchymal involvement by W-CD disease, with supportive ultrastructural findings. The synchronous W-CD flare and biopsy-proven FSGS and the rapid and sustained response of both to limited treatment suggest a causative association.

A 53-year-old man visited our hospital complaining of high fever. Chest computed tomography showed left pleural effusion and mediastinitis. He developed painful red subcutaneous nodules in his bilateral lower extremities. Thoracoscopy-assisted exploratory excision showed visceral pleura thickening; panniculitis in the periaortic area was histologically proven. The patient was treated with corticosteroid therapy which immediately reduced the fever. Subsequent imaging examinations after corticosteroid therapy showed improvement of mediastinitis and pleural effusion. This case reminds us that Weber-Christian disease (WCD) should be included in the differential diagnosis of mediastinitis although WCD is rarely associated with thoracic involvement.

Weber-Christian disease is a febrile, relapsing, non-suppurative panniculitis of unknown etiology. Lobular panniculitis is the essential feature in biopsy specimens and evolves through three recognizable stages. We report a case of Weber-Christian disease with bilateral orbital involvement, at different stages, affecting the orbital fat along with enophthalmos in one orbit, and the upper preaponeurotic fat pad in the other. Weber-Christian disease was refractory to treatment with conventional immunosuppressive regimens; however, early inflammatory-but not chronic fibrotic-orbital lesions responded dramatically to anti-tumor necrosis factor (TNF) therapy. A literature review revealed five additional cases of orbital Weber-Christian disease, none treated with anti-TNF antibodies. Of these, four presented initially with proptosis, representing early stages of inflammation, and two subsequently developed enophthalmos, representing late, inactive stage of the disease. Although orbital Weber-Christian disease is rare, ophthalmologists need to be aware of this entity. Depending on the stage of inflammation, Weber-Christian disease should be included in the differential diagnosis of both proptosis and enophthalmos. Anti-TNF antibodies can successfully treat patients at the early inflammatory stage., (Copyright © 2010 Elsevier Inc. All rights reserved.)

Weber-Christian Disease (WCD), also known as relapsing febrile lobular non-suppurative panniculitis, is a rare condition characterized by recurrent subcutaneous inflammatory nodules in the adipose tissue in addition to fever, malaise and other systemic manifestations such as polyarthralgia and polymyalgia. The association with small vessel vasculitis has been rarely reported. We report here an unusual case of WCD associated with small vessels vasculitis also describing the efficacy of Cyclosporin A treatment.

A 22-year-old male patient with Weber-Christian disease (WCD) presenting with periorbital swelling is a very rare occurrence. Lobular panniculitis in the periorbital and anterior tibial skin was diagnosed in this patient, based on the histological findings from biopsy specimens. The differential diagnosis was done before administering systemic corticosteroid. A dramatic response was achieved several days later, but relapse with intractable fever occurred 1 month later. The use of other immunosuppressive agents failed, and he died of septic shock 2 months later. The preceding infection may have been the immunopathologic factor in the development of WCD. Other effective treatments for WCD may be described in the future.

We report a case of Weber-Christian disease confirmed by skin biopsy in a patient who presented with collapsing glomerulopathy and lipophagic interstitial nephritis. On renal biopsy, glomerular visceral epithelial cells, tubular cells, and interstitial macrophages were loaded with inclusions that were morphologically consistent with oxidized lipoproteins, suggesting that lipids derived from the panniculitis may have an etiopathogenic role.

Background: The purpose of the present paper was to describe the clinical manifestations and treatment of patients with panniculitis., Methods: From January 1983 to December 2002, 4294 patients were treated for pediatric rheumatological diseases at Pediatric Rheumatology Unit, University of São Paulo, Brazil. Of these, 35 children and adolescents (0.8%) presented with panniculitis: erythema nodosum (EN) or Weber-Christian disease (WCD). Clinical characteristics, laboratory exams, biopsy of the lesion, treatment and clinical course were studied., Results: Of the 35 patients, 29 presented with EN and six with WCD, one of these with cytophagic histiocytic panniculitis. Mean age at symptom onset was 85 months (6-204 months) and the mean duration of follow up was 55 months (1-144 months). All the patients presented with inflammatory subcutaneous nodules. The patients with WCD presented with systemic manifestations and cutaneous atrophy. The principal etiologies of EN were streptococcal infection (42%), undetermined (13.5%), pulmonary tuberculosis (10%), and acute rheumatic fever (10%). Biopsy of the nodules indicated septal panniculitis in 14 patients with EN and lobular panniculitis without vasculitis in the patients with WCD, one of which had cytophagic histiocytic panniculitis. There was recurrence in 11 patients (38%) with EN and in all those with WCD. Non-steroidal anti-inflammatory drugs were used in 15 patients with EN and corticosteroids and/or immunosuppressive drugs in the six patients with WCD. Three patients died., Conclusions: 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.

We report a patient with multiple brain abscesses due to Staphylococcus cohnii. While these brain abscesses markedly responded to the antibiotics, this patient was subsequently suffered from subcutaneous inflammatory nodules in the adipose tissue, which diagnosed him as having Weber-Christian disease (WCD). This is the first report that subcutaneous inflammatory nodules in the adipose tissue, which lead the diagnosis of WCD, followed multiple brain abscesses. To our knowledge, S. cohnii has not yet been reported to cause multiple brain abscesses in humans. Although the etiology of WCD is unknown, an immune mechanism has been implicated in the pathogenesis. Therefore, we should notice that patients with WCD could be immunocompromised hosts with a higher risk to suffer from severe opportunistic infections.

Idiopathic nodular panniculitis is a condition characterized by the recurrent appearance of inflammatory nodules in the subcutaneous fat. In this report, an infant affected with Niemann-Pick disease and recurrent lobular panniculitis is described and discussed.

The aim of the paper is to present a case of a 5-year-old boy hospitalised because of recurrent high temperature and reddish blue infiltration of skin and hypodermis lasting 3 months. Because of pancytopenia and hepatomegaly, we suspected a proliferative disease of the haematopoietic or lymphatic system. Several bone marrow biopsies, did not show neoplastic changes. Serological tests excluded virus and animal borne infections. In the histopathologic examination of skin and hypodermis biopsy there was an inflammatory infiltration of the adipose tissue without features of vessel inflammation (the Weber-Christian syndrome). Al though steroid therapy was applied, the infiltration of the adipose tissue did not regress, while liver dysfunction symptoms intensified and pancytopenia and fever continued. The patient died with symptoms or respiratory and circulatory failure. This case of a 5-year-old boy is presented because of extreme diagnostic difficulties, which occurred at each phase of investigations. The repeated tests, as well as several verifications of the sampled histopathological material did not bring any unequivocal proof of the postulated diagnosis.

Cases of red fingers syndrome and nodular vasculitis in patients with hepatitis C have rarely been described. Both red fingers syndrome and nodular vasculitis are characterized by microscopic vasculitis, and it has been hypothesized that hepatitis C plays a role in the pathogenesis of cutaneous disorders such as vasculitis, especially in cryoglobulinaemic vasculitis. We describe the case of a 75-year-old woman diagnosed with chronic hepatitis C who presented with nodular lesions on her right thigh and red fingers syndrome. A skin biopsy taken from a nodule showed infiltration of the vessel walls by mononuclear cells and septal and lobular panniculitis. The diagnosis of nodular vasculitis was established. In our opinion, red fingers syndrome and nodular vasculitis might be related to a vascular reaction of immune-mediated mechanisms induced by hepatitis C.

The clinical and pathological findings in three unrelated weimaraners with pyrexia and multiple subcutaneous nodules are reported. Abdominal pain was an additional feature in two of the dogs and clinical investigations revealed inflammation of subcutaneous, mesenteric and falciform fat. Histopathological findings were consistent with pansteatitis. In the third dog, lesions were apparently limited to the subcutis and, hence, a diagnosis of nodular panniculitis was made. Microbiological examination of tissues was negative in all dogs, and there was no evidence of pancreatic disease. This report thus describes a presumed sterile and idiopathic panniculitis/pansteatitis complex in weimaraner dogs. Although the aetiology is unknown, this may represent an immune-mediated disorder.

Unlabelled: The second part of our review of panniculitis summarizes the clinicopathologic features of the mostly lobular panniculitides. Erythema induratum of Bazin (nodular vasculitis) represents the most common variant of lobular panniculitis with vasculitis, although controversy persists about the nature of the involved vessels. Mostly lobular panniculitides without vasculitis comprise a series of disparate disorders. These include sclerosing panniculitis that results from chronic venous insufficiency of the lower extremities; panniculitis with calcification of the vessel walls such as calciphylaxis and oxalosis; and inflammatory diseases with crystals within the adipocytes such as sclerema neonatorum, subcutaneous fat necrosis of the newborn, and poststeroid panniculitis. Connective tissue diseases, such as systemic lupus erythematosus and dermatomyositis, pancreatic diseases, and alpha(1)-antitrypsin deficiency may also show a mostly lobular panniculitis with characteristic histopathologic features. Lobular panniculitis may also be an expression of infections, trauma, or factitial causes involving the subcutaneous fat. Lipoatrophy refers to a loss of subcutaneous fat due to a previous inflammatory process involving the subcutis, and it may be the late-stage lesion of several types of panniculitis. In contrast, lipodystrophy means an absence of subcutaneous fat with no evidence of inflammation and often the process is associated with endocrinologic, metabolic, or autoimmune diseases. Finally, cytophagic histiocytic panniculitis is the term that has been used to describe two different processes: one is inflammatory, a lobular panniculitis, and the other one is neoplastic, a subcutaneous T-cell lymphoma. The only common feature of these two different processes is the presence of cytophagocytosis in the lesions. (J Am Acad Dermatol 2001;45:325-61.), Learning Objective: At the completion of this learning activity, participants should be familiar with the pathogenesis, clinical manifestations, histopathologic findings, and treatment options for the most frequent variants of the lobular panniculitides.

Weber-Christian disease is an inflammatory disorder of fatty tissue which usually presents with raised red tender nodules in the skin. Although there may be additional systemic upset, there are very few reports of neurological features associated with this condition. We report a patient with biopsy-confirmed systemic Weber-Christian disease in whom a transient partial myelopathy, of probable inflammatory origin, was the most prominent feature. Based on recent reports of the effects of immune mediators on neuronal function, a possible pathogenetic explanation for this syndrome is suggested.

Weber-Christian disease (WCD) is a rare inflammatory disease of adipose tissue, which is characterized by painful cutaneous nodules and constitutional symptoms. Although any area of the body containing fat can be affected by WCD, the involvement of retrobulbar fat is uncommon and proptosis is a rare presenting manifestation. We report a case who presented with proptosis of the right eye which is accompanied by painful subcutaneous nodules, high fever and myalgia. Biopsies of retrobulbar tissue and suprapubic nodule showed lobular panniculitis with mixed cellular infiltration, mainly composed of histiocytes and lymphocytes. He responded well to high-dose glucocorticoid.

Background: Weber-Christian disease is a controversial entity that histologically presents as a lobular panniculitis. A systemic variant of this disorder, in which visceral fat is affected, has been reported., Objective: A 29-year-old woman with Weber-Christian disease developed a rapidly enlarging abdominal mass during hospitalization for a flare of her skin condition. The mass resolved spontaneously. Radiographic studies demonstrated a mesenteric cyst, which was thought to represent involvement of mesenteric fat by Weber-Christian disease., Conclusion: The appearance of an abdominal or pelvic mass in a patient with Weber-Christian disease may be due to visceral involvement by the inflammatory process.

A case of 12 year-old Nigerian male is presented. He had an unusual variant of Weber-Christian disease and manifested massive subcutaneous indurations and nodules limited to the cheeks, lips, left pectoral, infraclavicular and supraclavicular areas. A wedge biopsy revealed suppurative granulomatous eosinophilic panniculitis. Despite exhaustive investigations, no obvious trigger of the panniculitis could be identified. Response to corticosteroids and to empirical trials with other drugs was poor, and the outcome was fatal. We believe this is the first report from Nigeria of this rare variant of Weber-Christian panniculitis in the paediatric age, and draw attention to the life-threatening nature of this disorder.

Purpose: To report an association between central serous retinopathy and Weber-Christian disease., Methods: A case report., Results: Central serous retinopathy was noticed in a patient with Weber-Christian disease possibly secondary to vasculitis involving the choriocapillaris., Conclusions: Central serous retinopathy may be associated with an uncommon systemic condition like Weber-Christian disease.

A 51-year-old Japanese woman had erythematous, tender subcutaneous nodules on her lower legs and buttocks, as well as fever, pancytopenia, and liver dysfunction. Histopathologic examination of the nodules showed subcutaneous T-cell lymphoma with cytophagocytosis and membranocystic lesions. Our patient was unique in having the combination of cytophagic histiocytic panniculitis due to subcutaneous T-cell lymphoma and membranocystic lesions.

A seven-year retrospective study was done on histologically proven nodular vasculitis presenting at the National Skin Centre, Singapore. We collected 25 patients and found that they differed from the classical European model. They most commonly involved the shins and did not ulcerate. A tuberculous aetiology accounted for 28% of cases. Useful indicators of tuberculosis were a past history of tuberculosis, an abnormal chest X-ray and a strongly positive Mantoux test.

Glucocorticoids have a strong anti-inflammatory action, and are indispensable in the treatment of inflammatory diseases. We had a patient with the Weber-Christian disease having an intractable high fever that did not respond to even a high-dose glucocorticoid therapy, but was responsive to a nonsteroidal antiinflammatory drug. To elucidate possible mechanisms of the glucocorticoid-unresponsive fever, we have investigated the in vitro production of two eicosanoids, prostaglandin (PG)E2 and leukotriene (LT)B4, from the peripheral blood polymorphonuclear leukocytes after stimulation by ionophore A23187. The patient's leukocytes produced much larger amount of PGE2, but the same amount of LTB4, as did those of two control groups. More interestingly, the production of eicosanoids was inhibited by dexamethasone less in the patients than in the controls. Indomethacin suppressed the production of PGE2 both in the patients and in the controls. These results might be relevant in the glucocorticoid-unresponsive pyrexia.

Weber-Christian disease, a disease of unknown aetiology, is characterised by relapsing febrile episodes and systemic panniculitis. Glucocorticoid therapy is often useful during acute phases of the disease. This report describes a patient in whom hyperpyrexia did not respond to high-dose glucocorticoid treatment, yet did respond to a non-steroidal anti-inflammatory drug (NSAID).

We describe a previously unreported association of idiopathic retroperitoneal fibrosis with mesenteric, pulmonary and perhaps periarticular fibrotic involvement, and panniculitis resembling Weber-Christian panniculitis. The onset of the disease was uncommon with arthritis. Then, several episodes of migratory panniculitis appeared; biopsy showed septal panniculitis without necrosis of adipose tissue. Considering the patient's anamnesis, it is tempting to speculate that these manifestations all originate from the same mechanism.

A case of 22-year-old man suffering from ulcerative colitis, in whom Weber-Christian panniculitis and musculitis occurred simultaneously, is reported. He had suffered from ulcerative colitis for 7 years and he again had bloody stools 1 month before being admitted. Two weeks before admission, large (2-4 cm) subcutaneous tumors appeared. Weber-Christian panniculitis, musculitis, and active stage ulcerative colitis were diagnosed. All the symptoms went into remission after steroid therapy. The possibility of the existence of a common antigen in enterobacterial wall, colonic epithelium, and adipose tissue was suggested.