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Rev Med Inst Mex Seguro Soc ; 58(3): 348-352, 2020 05 18.
Article Es | MEDLINE | ID: mdl-34002995

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.

Erythema Nodosum , Panniculitis, Nodular Nonsuppurative , Panniculitis , Vasculitis , Adult , Biopsy , Erythema Nodosum/complications , Erythema Nodosum/diagnosis , Female , Humans , Panniculitis/complications , Panniculitis/diagnosis , Panniculitis, Nodular Nonsuppurative/complications , Panniculitis, Nodular Nonsuppurative/diagnosis , Young Adult
BMJ Case Rep ; 12(5)2019 May 22.
Article En | MEDLINE | ID: mdl-31122958

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.

Panniculitis, Nodular Nonsuppurative/diagnosis , Aged , Diagnosis, Differential , Fever/etiology , Humans , Immunosuppressive Agents/administration & dosage , Immunosuppressive Agents/therapeutic use , Male , Mycophenolic Acid/administration & dosage , Mycophenolic Acid/therapeutic use , Panniculitis, Nodular Nonsuppurative/complications , Panniculitis, Nodular Nonsuppurative/drug therapy , Syndrome
Int J Rheum Dis ; 21(2): 573-578, 2018 Feb.
Article En | MEDLINE | ID: mdl-26224252

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.

Adrenal Cortex Hormones/therapeutic use , Methylprednisolone/therapeutic use , Multiple Pulmonary Nodules/drug therapy , Panniculitis, Nodular Nonsuppurative/drug therapy , Biopsy , Cyclophosphamide/therapeutic use , Drug Therapy, Combination , Humans , Immunosuppressive Agents/therapeutic use , Male , Middle Aged , Multiple Pulmonary Nodules/diagnosis , Multiple Pulmonary Nodules/etiology , Panniculitis, Nodular Nonsuppurative/complications , Panniculitis, Nodular Nonsuppurative/diagnosis , Positron Emission Tomography Computed Tomography , Treatment Outcome
Ultrastruct Pathol ; 36(6): 415-8, 2012 Dec.
Article En | MEDLINE | ID: mdl-23216240

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.

Glomerulosclerosis, Focal Segmental/etiology , Kidney/pathology , Panniculitis, Nodular Nonsuppurative/complications , Biopsy , Glomerulosclerosis, Focal Segmental/drug therapy , Glomerulosclerosis, Focal Segmental/pathology , Humans , Kidney/ultrastructure , Male , Microscopy, Electron , Middle Aged , Nephrotic Syndrome/etiology , Nephrotic Syndrome/pathology , Panniculitis, Nodular Nonsuppurative/drug therapy , Panniculitis, Nodular Nonsuppurative/pathology , Predictive Value of Tests , Steroids/therapeutic use
Intern Med ; 51(8): 943-7, 2012.
Article En | MEDLINE | ID: mdl-22504256

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.

Mediastinitis/diagnosis , Panniculitis, Nodular Nonsuppurative/diagnosis , Pleural Effusion/diagnosis , Pleurisy/diagnosis , Diagnosis, Differential , Humans , Male , Mediastinitis/etiology , Middle Aged , Panniculitis, Nodular Nonsuppurative/complications , Pleural Effusion/etiology , Pleurisy/etiology
Surv Ophthalmol ; 55(6): 584-9, 2010.
Article En | MEDLINE | ID: mdl-20701939

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.

Anti-Inflammatory Agents/therapeutic use , Antibodies, Monoclonal/therapeutic use , Orbital Diseases/complications , Orbital Diseases/drug therapy , Panniculitis, Nodular Nonsuppurative/complications , Panniculitis, Nodular Nonsuppurative/drug therapy , Adult , Diagnosis, Differential , Enophthalmos/diagnosis , Exophthalmos/diagnosis , Female , Humans , Infliximab , Orbital Diseases/diagnosis , Panniculitis, Nodular Nonsuppurative/diagnosis , Tomography, X-Ray Computed , Tumor Necrosis Factor-alpha/immunology
Rheumatol Int ; 30(6): 797-9, 2010 Apr.
Article En | MEDLINE | ID: mdl-19506878

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.

Cyclosporine/pharmacology , Panniculitis, Nodular Nonsuppurative/complications , Panniculitis, Nodular Nonsuppurative/drug therapy , Vasculitis/drug therapy , Vasculitis/etiology , Biopsy , Blood Vessels/drug effects , Blood Vessels/immunology , Blood Vessels/pathology , Child , Chronic Disease , Cyclosporine/therapeutic use , Humans , Immunosuppressive Agents/pharmacology , Immunosuppressive Agents/therapeutic use , Male , Panniculitis, Nodular Nonsuppurative/physiopathology , Recurrence , Skin/drug effects , Skin/immunology , Skin/pathology , Treatment Outcome , Vasculitis/physiopathology
Clin Rheumatol ; 26(6): 1002-4, 2007 Jun.
Article En | MEDLINE | ID: mdl-16550303

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.

Angioedema/diagnosis , Fever/etiology , Panniculitis, Nodular Nonsuppurative/complications , Panniculitis, Nodular Nonsuppurative/diagnosis , Panniculitis/etiology , Adult , Angioedema/etiology , Diagnosis, Differential , Fatal Outcome , Fever/drug therapy , Glucocorticoids/therapeutic use , Humans , Male , Methylprednisolone/therapeutic use , Panniculitis, Nodular Nonsuppurative/drug therapy , Shock, Septic/microbiology , Staphylococcal Infections
Am J Kidney Dis ; 48(3): 484-8, 2006 Sep.
Article En | MEDLINE | ID: mdl-16931223

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.

Glomerulonephritis/etiology , Panniculitis, Nodular Nonsuppurative/complications , Panniculitis, Nodular Nonsuppurative/pathology , Adult , Biopsy , Glomerulonephritis/pathology , Humans , Male , Recurrence
Pediatr Int ; 48(1): 48-53, 2006 Feb.
Article En | MEDLINE | ID: mdl-16490070

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.

Panniculitis , Adolescent , Child , Child, Preschool , Erythema Nodosum/complications , Erythema Nodosum/drug therapy , Female , Follow-Up Studies , Humans , Infant , Male , Panniculitis/complications , Panniculitis/drug therapy , Panniculitis/pathology , Panniculitis, Nodular Nonsuppurative/complications , Panniculitis, Nodular Nonsuppurative/drug therapy
J Eur Acad Dermatol Venereol ; 19(5): 600-2, 2005 Sep.
Article En | MEDLINE | ID: mdl-16164717

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.

Niemann-Pick Diseases/complications , Niemann-Pick Diseases/diagnosis , Panniculitis, Nodular Nonsuppurative/complications , Panniculitis, Nodular Nonsuppurative/diagnosis , Betamethasone/therapeutic use , Biopsy, Needle , Drug Therapy, Combination , Female , Follow-Up Studies , Fusidic Acid/therapeutic use , Humans , Immunohistochemistry , Infant , Niemann-Pick Diseases/therapy , Panniculitis, Nodular Nonsuppurative/drug therapy , Risk Assessment , Severity of Illness Index , Treatment Outcome
J Neurol Sci ; 238(1-2): 97-100, 2005 Nov 15.
Article En | MEDLINE | ID: mdl-16054652

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.

Brain Abscess/complications , Brain Abscess/microbiology , Panniculitis, Nodular Nonsuppurative/complications , Staphylococcal Infections/microbiology , Adipose Tissue/diagnostic imaging , Brain/pathology , Brain Abscess/diagnostic imaging , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Tomography, X-Ray Computed
Med Wieku Rozwoj ; 8(2 Pt 1): 201-8, 2004.
Article Pl | MEDLINE | ID: mdl-15738595

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.

Histiocytosis/diagnosis , Pancytopenia/etiology , Panniculitis, Nodular Nonsuppurative/diagnosis , Panniculitis/diagnosis , Adipose Tissue/pathology , Child, Preschool , Diagnosis, Differential , Fatal Outcome , Histiocytosis/complications , Histiocytosis/pathology , Humans , Male , Panniculitis/complications , Panniculitis/pathology , Panniculitis, Nodular Nonsuppurative/complications , Panniculitis, Nodular Nonsuppurative/pathology , Time Factors