Alves M F, Filgueira A L, Lorena D E, Porto L C
Service of Dermatology, Hospital Universitário Pedro Ernesto, State University of Rio de Janeiro, RJ, Brazil.
Am J Dermatopathol. 1998 Feb;20(1):41-7. doi: 10.1097/00000372-199802000-00008.
Cutaneous mucinoses are a heterogeneous group of diseases characterized by the focal or diffuse dermal deposition of glycosaminoglycans. The histopathologic examination of many cutaneous mucinoses reveals that the collagen fibers are fragmented. We wanted to characterize the type I (COL1) and type III (COL3) collagen distribution in skin biopsy specimens of patients with cutaneous mucinosis. The diagnosis of mucinosis was based on a modification of the classification by Rongioletti and Rebora: four patients had familial papulonodular mucinosis: four had papular mucinosis, one of which was associated with myxedema and one had scleromyxedema; and one had focal mucinosis. We performed anti-type I and type III collagens immunolabeling on frozen sections. Immunofluorescence for COL1 was increased in the superficial dermis of 2/4 familial papulonodular mucinosis, in 5/5 of papular mucinosis, and in scleromyxedema and focal mucinosis cases. The mid-dermis showed intense staining for COL1 at the periphery of collagen bundles and, in three cases of familial papulonodular mucinosis and two cases of papular mucinosis, a lacy appearance. The superficial dermis of familial papulonodular mucinosis specimens and of papular mucinosis + myxedema, scleromyxedema, and focal mucinosis specimens had decreased COL3 staining. The mid-dermis showed a more prominent fibrillar staining at the periphery of the collagen bundles, and two cases of papular mucinosis showed intense labeling for COL3. Both COL1 and COL3 distributions are altered in cutaneous mucinosis. An intense labeling with COL1 is predominantly found in the superficial layer of cutaneous mucinosis. Cases of FTP revealed decreased COL3 reactivity at the superficial layer.
皮肤黏蛋白病是一组异质性疾病,其特征是糖胺聚糖在真皮局灶性或弥漫性沉积。许多皮肤黏蛋白病的组织病理学检查显示胶原纤维断裂。我们想要描述皮肤黏蛋白病患者皮肤活检标本中I型(COL1)和III型(COL3)胶原的分布情况。黏蛋白病的诊断基于Rongioletti和Rebora分类法的改良:4例患有家族性丘疹结节性黏蛋白病;4例患有丘疹性黏蛋白病,其中1例与黏液性水肿相关,1例患有硬化性黏液性水肿;1例患有局限性黏蛋白病。我们对冰冻切片进行了抗I型和III型胶原免疫标记。在2/4的家族性丘疹结节性黏蛋白病、5/5的丘疹性黏蛋白病、硬化性黏液性水肿和局限性黏蛋白病病例中,COL1的免疫荧光在浅表真皮中增加。真皮中层在胶原束周围显示COL1强烈染色,在3例家族性丘疹结节性黏蛋白病和2例丘疹性黏蛋白病病例中呈花边样外观。家族性丘疹结节性黏蛋白病标本以及丘疹性黏蛋白病+黏液性水肿、硬化性黏液性水肿和局限性黏蛋白病标本的浅表真皮中COL3染色减少。真皮中层在胶原束周围显示更明显的纤维状染色,2例丘疹性黏蛋白病病例显示COL3强烈标记。皮肤黏蛋白病中COL1和COL3的分布均发生改变。在皮肤黏蛋白病的表层主要发现COL1强烈标记。家族性丘疹结节性黏蛋白病病例在表层显示COL3反应性降低。