Briceño-Moya Fernando, Hurtado-Díaz Jorge, Espinoza-Sánchez María Lucero
Instituto Mexicano del Seguro Social, Centro Médico Nacional La Raza, Hospital de Especialidades "Dr. Antonio Fraga Mouret", Departamento de Medicina Interna, Ciudad de México, México.
Rev Med Inst Mex Seguro Soc. 2020 May 18;58(3):348-352. doi: 10.24875/RMIMSS.M20000040.
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.
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.
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.
韦氏-克里斯蒂安病是一种复发性、非化脓性、发热性结节性脂膜炎，在年轻女性中更为常见。其特征为皮下结节反复发作，呈对称分布。病因不明，或继发于胰腺疾病、物理化学因素或α-1抗胰蛋白酶缺乏。它可累及任何有体脂的组织。组织学上为无血管炎的小叶性脂膜炎。
一名23岁女性。因盆腔、臀部和前臂出现皮下疼痛性结节入院，伴有红斑和色素沉着斑；右上眼睑瘀斑和双侧睑球粘连；体温38℃；多汗和口腔溃疡，无痛。血常规显示全血细胞减少，急性期反应物、淀粉酶升高，脂肪酶正常。血培养阴性。抗核抗体和补体正常。腹部计算机断层扫描显示皮下组织弥漫性高密度影。活检显示无血管炎的小叶性脂膜炎，符合韦氏-克里斯蒂安脂膜炎。患者接受了类固醇和免疫抑制治疗。
通过临床病程和辅助检查排除了感染性病因及其他引起红斑结节的原因。患者在数周内出现自发消退，有时病变会发展为无菌性脓肿，此外还有全身临床表现。皮质类固醇治疗被认为是成功的。
