硬皮病样慢性移植物抗宿主病

A case of sclerodermoid chronic graft-versus-host disease

报告1例硬皮病样慢性移植物抗宿主病。患者男,37岁。全身褐色斑块、鳞屑2年,伴溃疡及瘙痒2个月余。患者2011年10月28日于该院行其胞姐HLA6/6相合异基因造血干细胞移植。皮肤科检查:面部、躯干及四肢散在大小不一的褐色斑块,部分融合成大片状,其上可见点片状脱屑,触之稍硬,另散在色素减退斑;双小腿及骶尾部散在大小不一、形状不规则的浅溃疡,基底稍红,表面少许渗液;趾甲及双手拇指指甲变薄萎缩,颜色发白,部分缺失;双侧颊黏膜轻度萎缩及变薄。左小腿皮损组织病理学检查:表皮角化过度,棘层变薄,大片基底细胞液化变性,局部裂隙形成,皮突大部分消失;真皮全层胶原纤维均一化变性,嗜伊红性增强,血管附属器明显减少。根据患者临床及组织病理表现,诊断为硬皮病样慢性移植物抗宿主病。

A case of sclerodermoid chronic graft-versus-host disease（GVHD） is reported. A 37-year-old male presented with brown plaques and scales all over the body for 2 years, with ulcers and pruritus for more than 2 months. The patient received allogeneie hematopoietie stem cell transplantation in October 28, 2011. The donor was his sister. Physical examination showed partially merged firm brown plaques with some scales and scattered hypopigmentation on the face, trunk and limbs. Both lower legs and sacral region were covered with scattered inflamed ulcers in irregular shapes with secretion. Toenails and thumbnails were thinner and white, and partly lost. The buceal mueosa became atrophy and thinner. Histopathological examination of tissue from the left lower leg revealed hyperkeratosis, thinning of the stratum spinosum, liquefaction degeneration of basal cells, formation of focal fissures, and loss of most rete ridges. The dermal collagen fibers were degenerative homogeneously with more eosinophilic staining. The numbers of blood vessel and appendages were reduced. According to the clinical and pathogogical manifestations, the diagnosis of sclerodermoid chronic graft-versus-host disease was made.