Weber-Christian disease associated with the tip variant of focal segmental glomerulosclerosis: a case report.

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.

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

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.

Generalized chronic erythema nodosum: to consider Weber-Christian disease. / Eritema nudoso crónico de localización generalizada: a considerar enfermedad de Weber-Christian.

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.

INTRODUCCIÓN: La enfermedad de Weber-Christian es una paniculitis nodular recidivante, no supurativa, febril, más frecuente en las mujeres jóvenes. Se caracteriza por brotes recurrentes de nódulos subcutáneos, de distribución simétrica. Tiene un origen idiopático o es secundaria a enfermedad pancreática, agentes fisicoquímicos o déficit de alfa-1-antitripsina. Puede afectar cualquier tejido del organismo que tenga grasa. Histológicamente presenta paniculitis lobulillar sin vasculitis. CASO CLÍNICO: Mujer de 23 años que ingresó por nódulos subcutáneos dolorosos con equimosis en miembros pélvicos, glúteos y antebrazos, con placas eritematosas e hiperpigmentadas; equimosis en párpado superior derecho e hiposfagma bilateral; fiebre de 38 °C; diaforesis y úlceras orales no dolorosas. El hemograma mostró pancitopenia, elevación de los reactantes de fase aguda, y amilasa y lipasa normales. Los cultivos fueron negativos. Los anticuerpos antinucleares y el complemento fueron normales. La tomografía del abdomen presentó hiperdensidad en tejido graso subcutáneo diseminada. La biopsia reveló paniculitis lobulillar sin vasculitis, compatible con paniculitis de Weber-Christian. Se trató con esteroide e inmunosupresor. CONCLUSIONES: Se descartaron la etiología infecciosa y otras causas de nódulo eritematoso por la evolución clínica y los estudios complementarios. La paciente presentó en varias semanas una involución espontánea; en ocasiones, las lesiones evolucionaron a abscesos estériles, además de manifestaciones clínicas sistémicas. El tratamiento corticoideo tuvo buen resultado.

Pfeifer-Weber-Christian disease and successful treatment with mycophenolate mofetil: a case report.

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.

Weber-Christian disease presenting with lung nodules dramatically improved with corticosteroid therapy: one case report and literature review.

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 presenting with intractable fever and periorbital swelling mimicking angioedema.

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.

A case of Weber-Christian disease with collapsing glomerulopathy.

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.

Staphylococcus cohnii as a cause of multiple brain abscesses in Weber-Christian disease.

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.

Hemorrhagic diathesis associated with benign histiocytic, cytophagic panniculitis and systemic histiocytosis.

Five patients had a histiocytic, cytophagic panniculitis and a febrile, progressive course, which included hepatosplenomegaly, pancytopenia, liver function abnormalities, and a hemorrhagic death. The terminal hemorrhage was characterized by features of intravascular coagulation and liver failure. The histiocytosis could be found at times also in bone marrow, lymph nodes, liver and spleen, and serosal tissues, as well as in skin and subcutaneous tissue. Adequate biopsy diagnosis will prevent such cases from being labeled as Weber-Christian disease with bleeding, and offer an opportunity for appropriate treatment of this new syndrome of histiocytic, cytophagic panniculitis.

New observations in the histopathology of erythema nodosum.

Erythema nodosum presents variable clinical and histologic patterns of response in the vessels, septa, and fat lobules of the subcutaneous tissue. Acute or chronic phlebitis with hemorrhage may be commonly observed; acute panniculitis is observed in foci, but at time it may be the principal histologic feature; lymphocytic infiltration of fat lobules is often found, and lymphoid nodules are not infrequent; rarely, chronic granulomatous lesions involve septa or fat lobules; and proliferative lipocyte response may be observed. Because these variable histologic patterns may be coexistent, excision biopsy and multiple sections are necessary to recognize them. The variable histologic features correlate with the different clinical forms: acute nodular, chronic nodular, migrating plaque lesion, cellulitis, and the rate suppurative form. This variety of panniculus response in erythema nodosum can be explained on the basis of host-tissue response to a delayed hypersensitivity reaction to an antigenic stimulus.

Xanthogranuloma of the dura in systemic Weber-Christian disease.

A 51-year-old woman who had systemic Weber-Christian disease (WCD) displayed multiple xanthomatous lesions and a large xanthogranuloma of the tentorium cerebelli with signs of brainstem and cerebellar compression. Three other cases of dural xanthomatous lesions associated with systemic WCD have been reported. These dural and leptomeningeal xanthomatous lesions in WCD, a nonsuppurative inflammatory condition, are similar to those of Hand-Schüller-Christian disease, a quasi-neoplastic process.

Septal panniculitis as a manifestation of Lyme disease.

A 22-year-old woman presented with fever, chills, photophobia, and headaches, followed by a centrally clearing erythematous skin eruption, migratory polyarthralgias, conjunctivitis, and subsequently, tender, nodular skin lesions. Antibodies to Borrelia burgdorferi were consistent with acute Lyme disease. Skin biopsy revealed acute septal panniculitis. This dermatologic manifestation has not been previously described in Lyme disease.

Weber-Christian panniculitis with membranous glomerulonephritis.

A case of Weber-Christian panniculitis is described in which an acute phase of the disease was accompanied by the presence of circulating immune complexes, elevated serum levels of IgA and IgM, and membranous glomerulonephritis, with concomitant spontaneous remission of both diseases. This case reinforces the view that an immunologic dysfunction may be involved in the pathogenesis of Weber-Christian panniculitis.

Panniculitis associated with histoplasmosis and alpha 1-antitrypsin deficiency.

A patient with mediastinal histoplasmosis, alpha 1-antitrypsin deficiency, and panniculitis is presented. The patient showed dramatic response to treatment with ketoconazole. The associations of panniculitis with histoplasmosis and alpha 1-antitrypsin deficiency are discussed.

Sterile splenic abscesses in systemic Weber-Christian disease. Unique source of abdominal pain.

A patient with Weber-Christian disease and relapsing abdominal pain due to sterile splenic abscesses is presented. Histologically, acute and chronic inflammation with focal suppurative infiltrates of phagocytosed fat was detected in the absence of vascular or embolic disease, infection, or other apparent cause. Abdominal discomfort, an uncommon manifestation of Weber-Christian disease, usually is related to mesenteric panniculitis. Sterile splenic abscesses represent a previously undescribed complication of Weber-Christian disease, and another source of abdominal pain in this disorder. Although the precise pathophysiology of sterile splenic abscess formation in Weber-Christian disease is unknown, splenectomy is an effective means of alleviating pain.

Ten-year course of early-onset Weber-Christian syndrome with recurrent pneumonia: a suggestion for pathogenesis.

A surviving 10-year-old boy with infant-onset systemic Weber-Christian syndrome is reported. He has had recurrent episodes of fever, aseptic panniculitis, and pneumonia. Although corticosteroid therapy has succeeded, colchicine and non-steroidal anti-inflammatory drugs have failed to abort or prevent acute episodes. The persistent leukocytosis (even during remission), the recurrent episodes of fever with associated increase in the acute phase reactants, and the failure of the nonsteroidal anti-inflammatory drugs leads us to propose that this form of Weber-Christian syndrome reflects an inborn error in the regulation of the inflammatory response. Systematic investigation of this hypothesis could yield important information on the normal regulation of inflammation and could lead to a rational therapeutic approach to this puzzling and usually devastating illness.

Genetic study of a family with two members with Weber Christian disease (panniculitis) and alpha 1 antitrypsin deficiency.

Alpha 1 antitrypsin phenotypes and serum levels are presented for a family in which two brothers have Weber Christian disease and alpha 1 antitrypsin (PI) Z phenotypes. Clinical histories are described for these two men. A younger brother has the PI Z phenotype but does not have the disease, indicating that additional genetic and/or environmental factors contribute to the pathogenesis of Weber Christian disease. However, the two cases described provide additional evidence for a relationship between alpha 1 antitrypsin deficiency and the development of symptoms.

Musculoskeletal syndromes associated with malignancy.

Evidence has been presented supporting a causal relationship between malignancies and musculoskeletal syndromes. This discussion has dealt primarily with lesser known relationships, more common associations such as hypertrophic osteoarthropathy and dermatomyositis being reviewed elsewhere. The ones discussed herein closely mimic primary connective tissue diseases and offer an insight into the study of the pathogenesis of these primary diseases. In view of the natural history of malignant disease, the hope for such patients arises from the physicians early diagnosis and treatment of the underlying malignancy. Early diagnosis and treatment may in turn be entirely dependent on the physician's awareness of a musculoskeletal syndrome being the presenting feature of an otherwise occult neoplasm. Several connective tissue syndromes appear to predispose to the development of malignancy, and increasing evidence suggests that this development of malignancy may be further enhanced by immunosuppressive therapy.

Systemic nodular panniculitis with cardiac involvement.

A case of systemic nodular panniculitis is described in which the myocardium was found at necropsy to be extensively involved with focal interstitial carditis, identical histologically with nodules of panniculitis biopsied from the skin. This degree of myocardial involvement, which was not apparent during life and was not confined to pericardial or myocardial adipose tissue, has not previously been reported. The literature relating to nodular panniculitis is briefly reviewed and the concept of Weber-Christian disease critically re-appraised.