硬皮病样慢性移植物抗宿主病。7例分析。
Sclerodermatous chronic graft-versus-host disease. Analysis of seven cases.
作者信息
Chosidow O, Bagot M, Vernant J P, Roujeau J C, Cordonnier C, Kuentz M, Wechsler J, André C, Touraine R, Revuz J
机构信息
Department of Dermatology, Hôpital Henri-Mondor, Créteil, France.
出版信息
J Am Acad Dermatol. 1992 Jan;26(1):49-55. doi: 10.1016/0190-9622(92)70005-z.
BACKGROUND
Sclerodermatous chronic graft-versus-host disease is a disabling complication after allogeneic bone marrow transplantation from HLA-identical sibling donors. Only a few series of patients have been reported and the dermatologic features have never been extensively described.
OBJECTIVE
The purpose of the study was to describe clinical and biologic features of chronic sclerodermatous graft-versus-host disease and to compare them with scleroderma.
METHODS
We reviewed 196 patients grafted between April 1973 and July 1987 with survival times sufficient to be at risk of chronic graft-versus-host disease. Seven had the sclerodermatous form.
RESULTS
Most patients had disseminated sclerosis of the trunk and the proximal portions of the limbs. In two cases, atrophy of the skin was predominant, corresponding with a severe clinical evolution. Periorbital pigmentation was observed as an initial manifestation in three cases. Visceral manifestations resembled those observed in scleroderma but histologic and immunologic studies demonstrated clear differences. Response to therapy was variable.
CONCLUSION
Chronic sclerodermatous graft-versus-host disease may realize two different patterns. Major atrophy is associated with a more severe progression.
背景
硬皮病样慢性移植物抗宿主病是来自 HLA 相同同胞供者的异基因骨髓移植后的一种致残性并发症。仅有少数系列患者被报道，且其皮肤特征从未被广泛描述。
目的
本研究的目的是描述慢性硬皮病样移植物抗宿主病的临床和生物学特征，并将其与硬皮病进行比较。
方法
我们回顾了 1973 年 4 月至 1987 年 7 月间接受移植的 196 例患者，其生存时间足以使其有发生慢性移植物抗宿主病的风险。其中 7 例为硬皮病样形式。
结果
大多数患者躯干和四肢近端出现弥漫性硬化。2 例以皮肤萎缩为主，临床进展严重。3 例以眶周色素沉着作为初始表现。内脏表现与硬皮病所见相似，但组织学和免疫学研究显示出明显差异。对治疗的反应各不相同。
结论
慢性硬皮病样移植物抗宿主病可能呈现两种不同模式。严重萎缩与更严重的进展相关。
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