Santos Soraya Neves Marques Barbosa dos, Oliveira Gisele Viana de, Tavares André Luiz Pasqua, Massensini André Ricardo, Carvalhaes Lorenza Machado de Souza, Reljasvaara Ritva, Kitten Gregory Thomas
Hospital Semper, Belo Horizonte, Minas Gerais, Brazil.
Dermatol Online J. 2005 Mar 1;11(1):17.
Endostatin, an anti-angiogenic C-terminal fragment of collagen XVIII, has been recently reported to play a role in scleroderma pathogenesis, but collagen XVIII immunohistochemistry in scleroderma skin has still not been performed. Bullous scleroderma, a rare form of scleroderma, may have altered angiogenic and lymphangiogenic characteristics.
Our aim is to report a rare case of bullous scleroderma, studying the presence of fibronectin and collagens type I, III and XVIII in sclerodermic skin.
We describe the progression of bullous scleroderma in a 67-year-old patient since the first symptoms. Histological and immunohistochemical aspects of skin biopsies are compared to normal skin from a patient without scleroderma and are correlated with the pathogenesis of the disease. Indirect immunofluorescence measured by laser confocal microscopy allows quantitative determination of fibronectin and collagens type I, III and XVIII.
Dermo-epidermal cleavage, fibrosis and inflammation are the main histological findings. The dermal distribution and amounts of collagens and in the scleroderma patient are similar to normal skin. Conversely, both fibronectin and collagen XVIII are increased in scleroderma skin, suggesting their involvement in the pathogenesis of bullous scleroderma.
内皮抑素是胶原蛋白 XVIII 的一种抗血管生成 C 末端片段，最近有报道称其在硬皮病发病机制中发挥作用，但硬皮病皮肤的胶原蛋白 XVIII 免疫组化仍未进行。大疱性硬皮病是硬皮病的一种罕见形式，可能具有改变的血管生成和淋巴管生成特征。
我们的目的是报告一例罕见的大疱性硬皮病病例，研究硬皮病皮肤中纤连蛋白以及 I、III 和 XVIII 型胶原蛋白的存在情况。
我们描述了一名 67 岁患者自首次出现症状以来大疱性硬皮病的进展情况。将皮肤活检的组织学和免疫组化特征与一名无硬皮病患者的正常皮肤进行比较，并与疾病的发病机制相关联。通过激光共聚焦显微镜测量的间接免疫荧光可对纤连蛋白以及 I、III 和 XVIII 型胶原蛋白进行定量测定。
真皮 - 表皮分离、纤维化和炎症是主要的组织学发现。硬皮病患者皮肤中胶原蛋白的真皮分布和含量与正常皮肤相似。相反，硬皮病皮肤中的纤连蛋白和胶原蛋白 XVIII 均增加，提示它们参与了大疱性硬皮病的发病机制。
