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.

Angiopaniculitis granulomatosa mamaria / Mammary granulomatous angiopanniculitis

Introducción Las lesiones granulomatosas en la mama constituyen un variado espectro de alteracione que pueden traducir diferentes tipos de afecciones. Material y método: Presentamos una observación clinicopatológica acerca de una paciente de 49 años de edad que mostró lesiones recurrentes, primero en las mamas y más tarde en el muslo y pierna, que se resolvieron de forma espontánea sin ningún tratamiento. Resultados: EL examen citológico por punción de todas las lesiones mostró granulomas no necronizantes. El estudio histológico de la lesión mamaria evidenció una angiopaniculitis granulomatosa mamaria, consistente en la asociación de una angeítis llinfoide y una paniculitis granulomatosa no necronizante. Conclusión: La angiopaniculitis granulomatosa mamaria debe diferenciarse de las llamadas mastitis granulomatosas idiopáticas. El proceso puede simular clínica y radiográficamente un proceso neoplásico. Su curso evolutivo, a pesar de su carácter recurrente, es benigno, siendo considerada por algunos autores como una variedad de la enfermedad de Weber-Christian (AU)

Subarachnoid hemorrhage of unknown origin associated with Weber-Christian disease--case report.

A 58-year-old female, who had suffered from Weber-Christian disease for 26 years, presented with subarachnoid hemorrhage. Cerebral angiography showed dilatation of the basilar tip. An operation was performed in the chronic stage. The wall of dilated basilar artery was tough, but that of the right superior cerebellar artery was very thin and three small aneurysms were found on the right middle cerebral artery. Blood levels of fibrin degradation products, plasmin-alpha 2-plasmin inhibitor complex, and thrombin-antithrombin III complex were increased. The abnormality of the coagulation-fibrinolysis system and the fragility of the cerebral arteries related to Weber-Christian disease were probably the cause of the subarachnoid hemorrhage.

[Recurrent eyelid tumor in necrotizing panniculitis]. / Rezidivierender Lidtumor bei nekrotisierender Pannikulitis.

To our knowledge, necrotizing lid tumors occurring in the context of a necrotizing lobular panniculitis have not yet been described in the literature. Our patient, a 75-year-old male, presented with indolent, nonmoveable subcutaneous lumps that were centrally ulcerating. They appeared first on his upper lid, then on his lower lid and thereafter on the neck, back, upper extremity and abdomen, especially in the area of his cholecystectomy scar. A thorough work-up, which included repeated histopathological examinations performed by several laboratories, led to the diagnosis of an idiopathic necrotizing lobular panniculitis. A serum level determination performed later revealed no alpha 1-antitrypsin deficiency. As the eye-lid's subcutaneous tissue lacks fat, the association between the necrotizing lid tumors and the necrotizing panniculitis appears to be a paradox. In spite of the normal alpha 1-antitrypsin serum levels--determined when the patient's lesions had long ago healed--we think in retrospect that the differential diagnosis should have included the possibility of a decreased alpha 1-antitrypsin serum level (e.g. heterozygous MZ-phenotype). An alpha 1-antitrypsin level deficiency--with resulting decreased inhibition of collagenase and elastase--could account for the necrotizing process that also occurred in the eyelid's subcutaneous tissue. In the Van Gieson stain of this patient's eyelid biopsy, fragmentation of all visible collagen and elastic fibers was noted. In our opinion, the differential diagnosis does include lid involvement with secondary panniculitis caused by partially decreased alpha 1-antitrypsin serum levels and--by exclusion--idiopathic necrotizing lobular panniculitis. Therapeutic possibilities are briefly discussed.

Mesenteric panniculitis of the sigmoid colon. Report of two cases.

Mesenteric panniculitis is an extremely rare disease in which the normal fatty architecture of the mesentery is replaced by fibrosis, necrosis, and calcification. Grossly, the mesentery is massively thickened and rubbery, with irregular areas of reddish-brown to pale yellow foci resembling fat necrosis scattered throughout. Microscopically, inflammatory involvement of the fibroadipose tissue shows round cells, foam cells, and giant cells. Macrophages that have ingested fat, also called lipophages, are the hallmark of the diagnosis. One hundred twenty-two cases of mesenteric panniculitis have been described in the literature; only three of these involved the large-bowel mesentery. This paper presents a review of the literature and two new cases that involve exclusively the mesentery of the sigmoid colon.

Paniculite mesentérica: revisäo da literatura e apresentaçäo de um caso / Mesenteric panniculitis: review of the literature and report of a case

A paniculite mesentérica é uma patologia rara, de etiologia e patogenia ainda ignoradas. Relata-se um caso desta patologia salientando, e concordando com a literatura, a dificuldade diagnóstica pré e intraoperatória sendo a presença do tumor abdominal um dado clínico importante para o diagnóstico. O quadro anátomopatológico é característico com a presença dos lipófagos e a ausência de processo inflamatório. Quanto ao tratamento este permanece obscuro, visto que as doença apresenta uma evoluçäo invariavelmente benígna com ou sem tratamento complementar

Scrotal panniculitis in the prepuberal male patient.

A case of unilateral panniculitis with fat necrosis of subcutaneous scrotal fat tissue is reported. The typical presentation is that of unilateral or bilateral swelling, pain and a palpably tender mass in the scrotum located beneath the testicle. Conservative, non-operative treatment is successful.

[The so-called spontaneous nodular panniculitis (Weber-Christian lipodystrophy)]. / Sogenannte spontane herdförmige Pannikulitis (Weber-Christiansche Lipodystrophie)

Enlarged lymph nodes and a tumor, the size of a man's fist, were found in the abdomen of a female patient of 63. Histologic diagnosis determined a non-suppurative panniculitis with typical proliferation of foam cells and reactive inflammatory infiltration attaining the stage of granulomatosis. The disease is seen as granulomatous histiocytosis; a sure explanation of its pathogenesis is not yet possible.