Résumé
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.
Sujets)
Humains , Femelle , Adulte , Panniculite fébrile nodulaire récidivante non suppurée/induit chimiquement , Panniculite fébrile nodulaire récidivante non suppurée/anatomopathologie , Acide désoxycholique/effets indésirables , Mésothérapie/effets indésirables , Biopsie , Panniculite fébrile nodulaire récidivante non suppurée/traitement médicamenteux , Résultat thérapeutique , Derme/anatomopathologieRésumé
JUSTIFICATIVA E OBJETIVOS: A doença de Weber Christianou paniculite lobular idiopática é uma doença rara, que se apresenta com inflamação recorrente da camada adiposa da pele. O objetivo deste estudo foi descrever o quadro clínico dessa doença e informar sobre sua presença e impacto na qualidade de vida dos pacientes. RELATO DO CASO: Paciente do sexo feminino, portadora dessa doença, que ficou 20 anos sem diagnóstico, mas quando se detectou sua presença foi possível a remissão. CONCLUSÃO: A importância do caso descrito está em demonstrar como a falta de diagnóstico interfere na qualidade devida dos pacientes, portanto é bom lembrar que doenças raras acontecem e seu diagnóstico só é feito quando se pensam em hipóteses e diagnósticos diferenciais, quesitos esses indispensáveis para o exercício da clínica médica.
BACKGROUND AND OBJECTIVES: Weber Christian disease or idiopathic lobular pannicullitis is a skin condition that features recurring inflammation in the fat layer of the skin. The aim of this article is to describe this rare pathology, the clinical findings and remember your presence and their impact on patient's quality of life. CASE REPORT: Female patient, we report a case of patient with Weber Christian disease that was undiagnosed for 20 years and, when it was done it was possible to remission of the disease. CONCLUSION: The importance of the case described is to demonstrate how the lack of diagnosis interfere with the quality of life of patients and we always remember that rare diseases occurand their diagnosis is only made when thinking about chance and differential diagnosis, these questions essential to the exercise of clinical practice.
Sujets)
Humains , Femelle , Adulte , Médecine interne , Panniculite fébrile nodulaire récidivante non suppurée/diagnosticRésumé
Weber-Christian disease (WCD) is an inflammatory disease of subcutaneous fat tissue which is characterized by relapsing, febrile tender nodules and histologically lobular panniculitis. Any area of the body containing fat can be affected by WCD. Several cases of WCD have been reported as involvement of the heart, lung, liver and kidney. Acute illness (ex. acute myocardiac infarction, infection, etc) can be complicated with stress-related hyperglycemia and increase insulin restistance. Finally this case show that Weber-Christian disease accompanied by marked hyperglycemia and insulin resistance, which resolved soon after corticosteroid.
Sujets)
Humains , Coeur , Hyperglycémie , Infarctus , Insuline , Insulinorésistance , Rein , Foie , Poumon , Panniculite , Panniculite fébrile nodulaire récidivante non suppurée , Graisse sous-cutanéeRésumé
Weber Christian disease, an idiopathic relapsing febrile subcutaneous and visceral panniculitis is a rare disease in children. We report a case of Weber Christian disease for he first time from Kanti Children's Hospital.
Sujets)
Enfant , Femelle , Humains , Panniculite fébrile nodulaire récidivante non suppurée/diagnostic , Stéroïdes/usage thérapeutiqueRésumé
Weber-Christian disease is a rare inflammatory disease of subcutaneous fat necrosis that may or may not involve systemic disease. We report the results of a breast sonograph of a case diagnosed as a form of an acute lobular panniculitis affecting the breasts and upper limbs.
Sujets)
Région mammaire , Nécrose , Panniculite , Panniculite fébrile nodulaire récidivante non suppurée , Graisse sous-cutanée , Échographie , Membre supérieurRésumé
Weber Christian disease or idiopathic lobular panniculitis is a skin condition that features recurring inflammation in the fat layer of the skin. We report a case of a 17 year old boy with Weber Christian disease as its occurence is extremely rare with only sparse case reports available, who presented with eighteen-month history off fever with chills off and on, arthralgia predominantly of the knees, ankles, wrists and elbows without swelling or gross limitation of movement. There was also swelling around the eyes and puffiness of the face with lumpy swellings on the jaw, cheeks and chin and painful lumps on the anterior abdominal wall. On examination a febrile patient with pallor, pitting edema of the legs, periorbital edema and multiple non-matted firm to hard tender swellings of the face, jaw and chin and multiple firm tender lumps in the anterior abdominal wall was noted. Investigations revealed anemia, leucopenia, raised sedimentation rate and mild rise of liver enzymes. Biopsy of the abdominal nodule demonstrated a lobular panniculitis without vasculitis as seen in Weber Christian disease.
Sujets)
Adolescent , Azathioprine/usage thérapeutique , Humains , Immunosuppresseurs/usage thérapeutique , Mâle , Panniculite fébrile nodulaire récidivante non suppurée/traitement médicamenteuxRésumé
Weber-Christian disease (WCD) was first described by Pfeifer in 1892, and more clearly defined by Weber and Christian in the 1920s. It is a process of unknown etiology characterized by recurrent fever and inflammation of the adipose tissue. Pathological studies disclose areas of fat necrosis with an inflammatory infiltrate showing a lobular pattern and the usual presence of macrophages with foamy cytoplasm. The clinical signs include tender, palpable nodules, located mainly in the extremities, and fever, abdominal pain, arthritis and arthralgia and hepatosplenomegaly have also been reported. We present a case of Weber-Christian disease in which the presence of multiple subcutaneous nodules, enophthalmos, fatty liver, pericardial effusion was noticed. Biopsy of the skin showed mixed panniculitis in the subcutaneous fat layer. She responded well to glucocorticoid, colchicine and hydroxychloroquine.
Sujets)
Douleur abdominale , Tissu adipeux , Arthralgie , Arthrite , Biopsie , Colchicine , Cytoplasme , Énophtalmie , Membres , Stéatonécrose , Stéatose hépatique , Fièvre , Hydroxychloroquine , Inflammation , Macrophages , Panniculite , Panniculite fébrile nodulaire récidivante non suppurée , Épanchement péricardique , Peau , Graisse sous-cutanéeRésumé
Weber-Christian disease is an inflammatory disease of subcutaneous fat tissue which is characterized by relapsing, febrile tender nodules and histologically lobular panniculitis. Many cases originally considered as examples of Weber-Christian disease were later found to be other diseases when lobular panniculitis was reclassified, and some authors believe that Weber-Christian disease should no longer be considered as a distinctive entity. However, there are still occasional cases which cannot be diagnosed as other specific diseases but have the characteristic manifestations of Weber-Christian disease, and they attest to the fact that Weber-Christian disease could be a distinctive disease entity. We report here a case of Weber-Christian disease in a 22-year-old female who was treated successfully with steroid pulse therapy.
Sujets)
Femelle , Humains , Jeune adulte , Panniculite , Panniculite fébrile nodulaire récidivante non suppurée , Graisse sous-cutanéeRésumé
La paniculitis lobular idiopática, también denominada paniculitis nodular febril recurrente, o enfermedad de Weber-Christian, es el término empleado para describir un grupo de enfermedades que se manifiestan clínicamente por nódulos subcutáneos inflamatorios. Enfermedad de Weber-Christian es el término aplicado a los casos idiopáticos de paniculitis lobular asociada con síntomas sistémicos. Se presenta el caso de un paciente con síndrome febril muy intenso acompañado de lesiones en piel, las que aparecen sin causa alguna, y luego desaparecen sin tratamiento, asociado a varios síntomas sistémicos. Se detalla el caso y se hace una revisión de todo lo publicado en la literatura médica a propósito de esta enfermedad
Sujets)
Humains , Mâle , Adulte d'âge moyen , Érythème/étiologie , Panniculite fébrile nodulaire récidivante non suppurée/diagnostic , Panniculite fébrile nodulaire récidivante non suppurée/étiologie , Panniculite fébrile nodulaire récidivante non suppurée/physiopathologie , Rémission spontanée , Signes et symptômesRésumé
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.
Sujets)
Adulte , Humains , Mâle , Biopsie , Exophtalmie/anatomopathologie , Exophtalmie/étiologie , Exophtalmie/traitement médicamenteux , Glucocorticoïdes/administration et posologie , Imagerie par résonance magnétique , Panniculite fébrile nodulaire récidivante non suppurée/anatomopathologie , Panniculite fébrile nodulaire récidivante non suppurée/traitement médicamenteux , Panniculite fébrile nodulaire récidivante non suppurée/complicationsRésumé
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.
Sujets)
Adulte , Humains , Mâle , Biopsie , Exophtalmie/anatomopathologie , Exophtalmie/étiologie , Exophtalmie/traitement médicamenteux , Glucocorticoïdes/administration et posologie , Imagerie par résonance magnétique , Panniculite fébrile nodulaire récidivante non suppurée/anatomopathologie , Panniculite fébrile nodulaire récidivante non suppurée/traitement médicamenteux , Panniculite fébrile nodulaire récidivante non suppurée/complicationsRésumé
This is a report of a case, 7 1/2 year-old-boy having chronic febrile and recurrent crops of painful subcutaneous nodules on lower extremities, which had previously been diagnosed as Weber-Christian disease, which progressed to have cytophagic histiocytic activity in the skin, bone marrow with abnormal liver function and hemorrhagic diathesis. He was subsequently treated with corticosteroid without good response. After he was diagnosed as having cytophagic histiocytic panniculitis, cyclosporin A was administered intravenously in an initial dosage of 1 mg/kg/day and in oral maintenance dose of 10 mg/kg/day with a successful response and the patient completely recovered within 6 months with mild hypertension as an adverse effect.
Sujets)
Enfant , Ciclosporine/usage thérapeutique , Érythrocytes/immunologie , Stéatonécrose/traitement médicamenteux , Histiocytose/traitement médicamenteux , Humains , Mâle , Panniculite fébrile nodulaire récidivante non suppurée/traitement médicamenteux , PhagocytoseRésumé
A fatal case of cytophagic panniculitis is reported in a 9 year old Omani girl. The patient died of generalised haemorrhage and liver failure. The pathogenesis of the condition is discussed
Sujets)
Humains , Femelle , Histiocytes/anatomopathologie , Panniculite fébrile nodulaire récidivante non suppurée , Pancytopénie , Foie/physiopathologie , Maladies lymphatiquesRésumé
Se presenta el primer reporte en Uruguay de un caso de Paniculitis nodular no supurativa, o síndrome de Weber Christian. Esta es una enfermedad rara más aún en niños, y su etiología es desconocida. Esta se caracteriza clínicamente por nódulos subcutáneos, fiebre, repercusión general y manifestaciones sistémicas, sobre todo a nivel hepático, pulmonar y digestivo. Su diagnóstico es de sospecha clínica y confirmación anatomopatológica, con la biopsia de piel que evidencia una paniculitis lobulillar no supurativa característica. Se acompaña en ocasiones de alteraciones de la inmunidad celular. El tratamiento con corticoides es de relativo éxito, siendo el pronóstico incierto
Sujets)
Humains , Mâle , Nourrisson , Panniculite fébrile nodulaire récidivante non suppurée , Uruguay , Panniculite fébrile nodulaire récidivante non suppurée/diagnostic , Panniculite fébrile nodulaire récidivante non suppurée/anatomopathologie , Panniculite fébrile nodulaire récidivante non suppurée/thérapieRésumé
Os autores relatam um caso de amiloidose cutânea, localizada em uma paciente de 58 anos, associada a telangiectasia hemorrágica hereditária. Esta forma de amiloidose é rara, e apresenta as características histopatológicas e prognósticas diferentes das variantes liquenóide e maculosa. O aspecto peculiar deste caso é associaçäo das doenças, fato do nosso conhecimento näo foi referido na literatura
Sujets)
Humains , Femelle , Adulte d'âge moyen , Amyloïdose/complications , Amyloïde/métabolisme , Panniculite fébrile nodulaire récidivante non suppurée/complications , Télangiectasie hémorragique héréditaire/complications , Diabète/étiologieRésumé
Weber-Christian disease is an inflammatory disease of subcutneious fat tissue which is clinically characterized by relapsing, febrile, nodular, nonsuppurative pannicutilitiy. The cause is unknown, but the disease has been attributed to an autoimmune reaction, infectioris, halogen compounds, sarcoidosis, SLE and a-antitrypsin deficiency. We report here in a case of Weber-Christian disease in a 8-year-old girl, who had indurated erythemataus subcutaneous nodules on the extremities, buttock and trunk accompanied by various general symptoms such as fever, malaise, arthralgia and edema of the abclorren and legs. This case was successfully treated with dapsone,
Sujets)
Enfant , Femelle , Humains , Arthralgie , Fesses , Dapsone , Oedème , Membres , Fièvre , Jambe , Panniculite fébrile nodulaire récidivante non suppurée , SarcoïdoseRésumé
Un niño de 14 meses de edad ingresó por presentar placa indurada en la piel del muslo izquierdo y fiebre alta de un mes de evolución. Durante su hospitalización aparecieron nódulos subcutáneos sensibles con características inflamatorias en cara y tronco, adenopatías cervicales y hepatomegalia. En la biopsia de la piel había extensa destrucción del tejido celular subcutáneo, células espumosas, vasculitis e infiltración por leucocitos polimorfonucleares; en la de ganglio linfático se comprobó proliferación histiocitaria y en una muestra de hígado obtenida por punción se encontró evidencia de esteatosis. Se descartaron osteoartritis, celulitis, septicemia, tuberculosis, enfermedad del colágeno y neoplasia linforreticular. Recibió tratamiento con prednisona con excelente respuesta. Se ha mantenido asintomático a lo largo de 12 meses de seguimiento
Sujets)
Panniculite fébrile nodulaire récidivante non suppurée/anatomopathologie , Biopsie , Diagnostic différentiel , Panniculite fébrile nodulaire récidivante non suppurée/traitement médicamenteux , Prednisone/usage thérapeutiqueRésumé
No abstract available.
Sujets)
Panniculite , Panniculite fébrile nodulaire récidivante non suppuréeRésumé
Se reporta el caso de un paciente de 37 años, quien consultó por abdominal y otros síntomas inespecíficos, realizandose el diagnóstico de Paniculitis Mesentérica mediante el estudio histológico de un segmento de epiplón. Se revisan las caracteísticas clínicas de la Paniculitis Mesentérica y su diferencia con la Enfermedad de Weber-Christian