Winkelmann R K, McEvoy M T, Peters M S
Department of Dermatology, Mayo Clinic, Rochester, MN 55905.
J Am Acad Dermatol. 1989 Nov;21(5 Pt 1):971-8. doi: 10.1016/s0190-9622(89)70285-1.
The cases of three patients with an atrophic lipophagic granulomatous lipoatrophy are described and compared with 14 similar cases in the literature. In affected children erythematous lesions of the arms and legs usually occur, after which subcutaneous atrophy develops. Fever and edema are common. The histologic feature is a replacement of the fat lobule by lipophagic histiocytes and giant cells. No vasculitis or phlebitis is present. Previously, such cases have been called Weber-Christian disease or Rothmann-Makai syndrome, but they are best characterized by the distinctive clinical lesions, the course, and histologic findings. Corticosteroids apparently have been effective in some patients.
本文描述了3例萎缩性脂肪吞噬性肉芽肿性脂肪萎缩患者的病例,并与文献中14例类似病例进行了比较。在患病儿童中,手臂和腿部通常会出现红斑性病变,随后出现皮下萎缩。发热和水肿很常见。组织学特征是脂肪小叶被脂肪吞噬性组织细胞和巨细胞取代。不存在血管炎或静脉炎。以前,此类病例被称为韦伯-克里斯蒂安病或罗特曼-马凯综合征,但它们的最佳特征是独特的临床病变、病程和组织学表现。皮质类固醇在一些患者中显然有效。