Institute of Animal Pathology, Vetsuisse Faculty University Berne, Switzerland.
Vet Pathol. 2010 Mar;47(2):346-50. doi: 10.1177/0300985809358039. Epub 2010 Jan 28.
Scleromyxedema--the generalized form of lichen myxedematosus, a primary mucinosis--is a rare disease in human patients. It is characterized by dermal mucin deposits, increased numbers of fibroblasts, and variable fibrosis in the absence of thyroid disease. It is accompanied in 80% of cases by a monoclonal gammopathy. To date, scleromyxedema with systemic involvement has not been documented in domestic animals. This is the first report of a scleromyxedema-like syndrome in a cat, which had a substantial deposition of mucin in the dermis of the head and paws with a mild gammaglobulinemia of 2.25 g/dl (reference range, 1.39-2.22 g/dl). At necropsy, multiple nodules of connective tissue intermingled with mucin deposits were conspicuous on the surface of thoracic and abdominal organs. Such severe systemic accumulations of mucin have not been reported in human or veterinary medicine.
硬肿性黏液水肿——苔藓样黏蛋白病的全身性形式,是一种原发性黏蛋白病——在人类患者中较为罕见。其特征为皮肤黏蛋白沉积、成纤维细胞数量增加以及在无甲状腺疾病的情况下出现可变纤维化。在 80%的病例中,它伴随着单克隆丙种球蛋白血症。迄今为止,在宠物中尚未有全身性受累的硬肿性黏液水肿的记录。这是首例猫硬肿性黏液水肿样综合征的报告,其头部和足部皮肤有大量黏蛋白沉积,球蛋白血症为 2.25 g/dl(参考范围,1.39-2.22 g/dl)。尸检时,在胸腹部器官表面可见到与黏蛋白沉积混杂在一起的多个结缔组织结节。在人类或兽医医学中,尚未有如此严重的全身性黏蛋白积聚的报道。