Case for diagnosis. Noninfectious suppurative panniculitis induced by mesotherapy with deoxycholate.

A 28-year-old white female patient presented with multiple erythematous-to-violaceous, painful, suppurative nodules on the buttocks and thighs that appeared after two weeks of mesotherapy with deoxycholate, caffeine, sunflower liposomes, and sinetrol for localized fat. She was treated for atypical mycobacteriosis, but with no satisfactory response after antibiotic therapy. Bacterial, mycobacterial, and fungal culture were all negative. Histopathologic examination of the biopsy showed noninfectious suppurative panniculitis. It resolved after treatment with methotrexate, prednisone, and hydroxychloroquine. This report highlights the rarity of this complication, the importance of its early recognition, and differentiation with atypical fast growing mycobacterioses.

Case for diagnosis. Noninfectious suppurative panniculitis induced by mesotherapy with deoxycholate

Abstract A 28-year-old white female patient presented with multiple erythematous-to-violaceous, painful, suppurative nodules on the buttocks and thighs that appeared after two weeks of mesotherapy with deoxycholate, caffeine, sunflower liposomes, and sinetrol for localized fat. She was treated for atypical mycobacteriosis, but with no satisfactory response after antibiotic therapy. Bacterial, mycobacterial, and fungal culture were all negative. Histopathologic examination of the biopsy showed noninfectious suppurative panniculitis. It resolved after treatment with methotrexate, prednisone, and hydroxychloroquine. This report highlights the rarity of this complication, the importance of its early recognition, and differentiation with atypical fast growing mycobacterioses.

[Weber-Christian's disease: a preleukemic disorder?] / La maladie de Weber-Christian: s'agit-il d'un état pré-leucémique?

Weber christian's disease or idiopathic panniculitis is a rare condition characterized by an inflammation of the subcutaneous adipose tissue; it is a non-specific pathological condition that remains a central issue of debate and whose outcome is unpredictable. We here report the case of a 9-month-old female infant admitted in the hospital with sepsis preceded by respiratory symptoms and followed by the occurrence of small, hard, susceptible, asymmetrical erythematous subcutaneous nodes located in the arms and lower limbs. Cutaneous biopsy showed lobular hypodermitis with polymorphonuclear neutrophil infiltrations. The diagnosis of Weber Christian's disease was retained after eliminating other differential diagnoses. The patient was treated with prednisone with good outcome; during the follow-up period and during disease regression the diagnosis of B-cell acute lymphoblastic leukemia was made; this uncommon outcome has never been described in the literature. The aim of this study is to discuss any similar situation where another diagnosis was made in patients initially treated for idiopathic lobular panniculitis. This highlights the need for exhaustive etiological investigations and prolonged monitoring in order to search for a possible associated disorder.

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.

Ocular Inflammation: Can It Be a Sign of Activity of Weber-Christian Disease? A Case Report and Review of Literature.

PURPOSE: To present a case of Weber-Christian disease with symptomatic ocular involvment. Weber-Christian disease is a relapsing febrile nodular nonsuppurative panniculitis. It is characterized by malaise and fever accompanied by subcutaneous inflammatory nodules on the trunk and extremities. It can affect several organs, but ocular signs have been infrequently described in literature. METHODS: A 20-year-old woman with Weber-Christian disease presented with severe bilateral ocular inflammation. A complete ophthalmologic examination was performed. RESULTS: Visual acuity was 20/100 in both eyes and slit-lamp examination showed bilateral iridocyclitis. Bilateral cortico-nuclear cataract did not allow funduscopy and she underwent cataract extraction. Retinal vasculitis was detected. CONCLUSIONS: Patients with Weber-Christian disease can develop severe ocular inflammation. A complete ophthalmolgic examination should be done in these patients, especially when ocular involvement is the main sign of the disease. A further understanding of the severity of ocular inflammation proved fundamental in the management of the disease.

Weber-Christian disease with ileocolonic involvement successfully treated with infliximab.

Weber-Christian disease (WCD) is an inflammatory disease whose main histological feature is lobular panniculitis of adipose tissue. The location of panniculitis determines the clinical presentation, being the subcutaneous adipose tissue the most frequent one, followed by liver, spleen, bone marrow and mesenteric adipose tissue. Systemic corticosteroids are first line treatment, but other options should be considered if systemic symptoms are observed or in case of refractory clinical situation. We report herein a case with WCD showing orbital, mesenteric and ileocolonic involvement, which required surgical treatment and also developed postoperative recurrence. Symptoms were resolved by administration of thalidomide and, afterwards, infliximab. To our knowledge, this is the first report of Weber-Christian disease with luminal ileocolonic involvement, treated with infliximab.

[Weber-Christian disease].

In pursuance of the Federal law "On the fundamentals of health protection in RF", the Russian Ministry of Health approved the list of so-called orphan diseases, i.e. rare pathologies of great medical and social significance due to their life-threatening nature and immense burden on health budget. Weber-Christian disease is a chronic recurrent orphan diseases characterized by formation of painful dense nodes in subcutaneous fat free from suppuration and accompanied by episodic temperature changes, chill, muscular pain and sometimes damage to internal organs. The disease recurs at irregular intervals.

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.

[Lipoatrophic panniculitis in adults: treatment with hydroxychloroquine]. / Panniculite lipoatrophiante de l'adulte : traitement par hydroxychloroquine.

BACKGROUND: Lipoatrophic panniculitis is generally considered to be a rare disease affecting children. We report a case involving this condition in an adult patient presenting with striking clinical features and responding to hydroxychloroquine therapy. We discuss the nosological relationship between lipoatrophic panniculitis and connective tissue panniculitis. PATIENTS AND METHODS: A 62-year-old woman was referred to our institution with a six-month history of painful erythematous nodules and plaques on the calves, thighs, buttocks, breasts, abdomen and arms. With each outbreak of new lesions, she felt unwell and experienced fever, chills and sweating. After a few weeks, the lesions progressed circumferentially and led to large areas of subcutaneous atrophy showing a central depression covered with a yellowish, supple skin and surrounded by an annular, infiltrated, erythematous and tender inflammatory margin. Deep subcutaneous biopsy specimens showed typical features of lobular and septal lipophagic panniculitis, with a dense inflammatory infiltrate composed of large histiocytes, multinucleated giant cells and few neutrophils, without vasculitis. The patient was started on hydroxychloroquine 400 mg daily. Three weeks later, her pain and tenderness had completely resolved and the inflammatory margin of the lesions had clearly regressed. DISCUSSION: This unique condition, with distinctive clinical and histological features, is similar to cases described under the term "lipophagic panniculitis", seen mostly in children, but also "connective tissue panniculitis". Their clinical resemblance and response to hydroxychloroquine therapy leads us to think that these two entities, previously subsumed under the eponym of Weber-Christian disease or Rothman-Makai syndrome, are closely related. CONCLUSION: Dermatologists and dermatopathologists should be made aware of this unusual entity, and of the fact that it can arise in adult patients, so that they may make an early diagnosis and thus prevent the unsightly consequences of lipoatrophy.

Unusual systemic signs in a dog with sterile neutrophilic-macrophagic lymphadenitis and nodular panniculitis.

A 7 mo old female English springer spaniel was presented with diarrhea, vomiting, apathy, and hyperthermia. Further examinations revealed generalized lymphadenomegaly consistent with sterile neutrophilic-macrophagic lymphadenitis and pulmonary involvement. Subcutaneous nodules developed one day after presentation. Histology was consistent with sterile idiopathic nodular panniculitis and vasculitis. No infectious organism was isolated. The dog responded to prednisolone, but relapsed during medication tapering. Cyclosporine had to be added to control the disease. No further relapse had occurred 98 wk after the first presentation. This is an unusual presentation of a systemic sterile neutrophilic-macrophagic lymphadenitis with nodular panniculitis and vasculitis associated with gastrointestinal and pulmonary signs.

Orbital lobular panniculitis in Weber-Christian disease: sustained response to anti-TNF treatment and review of the literature.

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.

A patient with Pfeifer-Weber-Christian disease--successful therapy with cyclosporin A: case report.

BACKGROUND: Pfeifer-Weber-Christian disease (PWCD) is a rare inflammatory disorder of the subcutaneous fatty tissue. The diagnosis and therapy of this rare type of panniculitis is still controversial and will be discussed in this article. CASE PRESENTATION: We here report the rare case of a 64-year old male patient, with PWCD. The patient suffered from rheumatoid arthritis for several years, but then developed relapsing fever and recently occurring painful subcutaneous nodules predominantly at the inner part of his left upper limb with no signs of synovitis. Finally, a biopsy from one of the nodules revealed lobular panniculitis with mixed cell infiltrate, which was conformable only with PWCD, after excluding several differential diagnoses. In our patient PWCD developed despite immunosuppressive therapy with steroids and different disease modifying drugs, which the patient received to treat his underlying rheumatoid arthritis. However, when DMARD therapy was switched to Ciclosporin A the patient's symptoms resolved. CONCLUSION: Our observation supports the hypothesis that T cells are involved in the pathogenesis of PWCD. Thus, T cell modifying drugs should be primarily used to treat patients with this rare disorder.

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.

Childhood Weber-Christian disease: clinical investigation and virus detection.

OBJECTIVE: To investigate clinical features of childhood Weber-Christian disease (WCD) and the relationship between WCD and virus. METHODS: The clinical characteristics, laboratory findings, therapy and clinical course of nine patients were reviewed and the viral antigens (EBV, CMV, HSV1 and HSV2) were detected. RESULTS: They were six males and three females with a median age of 9 years. They included four simple and five systemic WCD, and all presented fever and tender subcutaneous nodules. Other clinical features included hepatomegaly in nine patients, splenomegaly in two, lymphadenhypertrophy in six, respiratory system involved in two, gastrointestinal tract involved in two and convulsion and bleeding in one. Anaemia was noted in seven patients, raised ALT in three, hypergammaglobulnaemia in three, proteinuria in one and three of six patients positive of antinuclear antibodies (ANA). No viral antigens were found. Corticosteroids therapy was administrated for all patients, immunoglobulin for two, non-steroidal anti-inflammatory agents for three and cyclophosphamide for one. Five patients released after therapy and three patients died. CONCLUSIONS: WCD is a severe disease characterized by relapsing, febrile subcutaneous nodules and biopsy is needed for early diagnosis. The viral infection may not be associated with WCD.

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 Rothmann-Makai panniculitis successfully treated with tetracycline.

Rothmann-Makai syndrome (lipogranulomatosis subcutanea) is a rare variant of Weber-Christian disease, but lacks visceral involvements and systemic manifestations. We herein report the case of a 56-year-old Chinese woman with this disorder who complained of subcutaneous tender nodules over her extremities, trunk, and face of 2 years' duration. She was firstly treated with 300 mg of oral roxythromycin for 8 weeks and subsequently treated with 400 mg of oral clarithromycin for the next 8 weeks. However, no significant effect was observed. She was then administered 200 mg of oral minocycline hydrochloride. After 1 week, her skin symptoms rapidly improved and no relapse has been observed in a follow-up period of 3 months. Considering that tetracycline antibiotics, but not macrolide antibiotics, inhibit the activity of pancreatic lipase in vitro, the efficiency of tetracycline antibiotics may be a clue to clarifying the pathogenesis of this disorder.