Sakuraoka K, Tajima S, Nishikawa T
Department of Dermatology, Keio University, School of Medicine, Tokyo, Japan.
Dermatology. 1992;185(3):196-201. doi: 10.1159/000247446.
Five cases of progressive facial hemiatrophy (PFH) are reported. A nonindurated depression on normal-colored skin was observed in the cheeks of 3 subjects, and 2 patients showed indurated, pigmented atrophic lesions associated with linear scleroderma or generalized morphea. Lipoatrophy with mild subcutaneous fibrosis was observed histologically in the patients with nonindurated depressions. In contrast, the patients with indurated lesions exhibited a marked dermal fibrosis and the disappearance of appendices in the dermis. When compared with unaffected skin used as a control, collagen and glycosaminoglycan contents were not different in diseased areas. However, the dermatan sulfate/hyaluronic acid ratio was increased 1.5- to 3.2-fold in PFH patients regardless of their clinical and histological differences. These results suggest that both types of PFH may be based on a similar connective tissue disorder.
报告了5例进行性面部半侧萎缩（PFH）病例。在3名受试者的脸颊上观察到正常肤色皮肤上有非硬结性凹陷，2名患者表现出与线状硬皮病或泛发性硬斑病相关的硬结性、色素沉着性萎缩性病变。组织学观察发现，非硬结性凹陷患者存在脂肪萎缩伴轻度皮下纤维化。相比之下，硬结性病变患者表现出明显的真皮纤维化和真皮附属器消失。与用作对照的未受影响皮肤相比，病变区域的胶原蛋白和糖胺聚糖含量没有差异。然而，无论临床和组织学差异如何，PFH患者的硫酸皮肤素/透明质酸比值增加了1.5至3.2倍。这些结果表明，两种类型的PFH可能都基于相似的结缔组织疾病。
