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[硬皮病样慢性移植物抗宿主病1例罕见病例。体外光化学疗法(ECP)成功治疗]

[A rare case of a sclerodermoid chronic graft versus host disease. Successful treatment with extracorporeal photopheresis (ECP)].

作者信息

Jung A G, Bertsch H P, Schoen M P, Lippert U

机构信息

Klinik für Dermatologie, Venerologie und Allergologie/Immunologisches Zentrum, Städtisches Klinikum Dessau, Auenweg 38, 06847, Dessau, Germany.

出版信息

Hautarzt. 2010 Jun;61(6):514-7. doi: 10.1007/s00105-010-1924-9.

DOI:10.1007/s00105-010-1924-9
PMID:20309512
Abstract

A 31-year-old woman presented with progressive deep linear induration on her lower abdomen, forearms and thighs. These symptoms developed three years after allogenic stem cell transplantation. Furthermore, the patient showed multiple hypopigmented lichenoid papules on the extensor surfaces of the forearms consistent with lichen sclerosus. Histological analysis of a biopsy specimen from her left thigh showed dermal sclerosis extending into the fascia, thus establishing the diagnosis of a rare combination of superficial and deep sclerodermoid chronic graft-versus-host disease. After 7 cycles of extracorporeal photopheresis, a marked resolution of the indurations and a reduction of the modified Rodnan skin score from 12 to 7 were noted.

摘要

一名31岁女性患者,下腹部、前臂和大腿出现进行性深部线性硬结。这些症状在异基因干细胞移植三年后出现。此外,患者前臂伸侧有多个色素减退的苔藓样丘疹,符合硬化性苔藓表现。对其左大腿活检标本进行组织学分析,显示真皮硬化延伸至筋膜,从而确诊为浅表和深部硬皮病样慢性移植物抗宿主病这一罕见组合。经过7个周期的体外光化学疗法后,硬结明显消退,改良罗德南皮肤评分从12分降至7分。

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A multicenter prospective phase 2 randomized study of extracorporeal photopheresis for treatment of chronic graft-versus-host disease.一项关于体外光化学疗法治疗慢性移植物抗宿主病的多中心前瞻性2期随机研究。
Blood. 2008 Oct 1;112(7):2667-74. doi: 10.1182/blood-2008-03-141481. Epub 2008 Jul 11.
2
U.K. consensus statement on the use of extracorporeal photopheresis for treatment of cutaneous T-cell lymphoma and chronic graft-versus-host disease.英国关于使用体外光化学疗法治疗皮肤T细胞淋巴瘤和慢性移植物抗宿主病的共识声明。
Br J Dermatol. 2008 Apr;158(4):659-78. doi: 10.1111/j.1365-2133.2007.08415.x. Epub 2008 Jan 30.
3
MRI findings in deep and generalized morphea (localized scleroderma).
深部和泛发性硬斑病(局限性硬皮病)的磁共振成像表现
AJR Am J Roentgenol. 2008 Jan;190(1):32-9. doi: 10.2214/AJR.07.2163.
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High-frequency ultrasound of skin involvement in systemic sclerosis reflects oedema, extension and severity in early disease.系统性硬化症皮肤受累的高频超声反映了疾病早期的水肿、范围及严重程度。
Rheumatology (Oxford). 2008 Jan;47(1):84-7. doi: 10.1093/rheumatology/kem307.
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A single-center pilot validation study of a new chronic GVHD skin scoring system.一项关于新型慢性移植物抗宿主病皮肤评分系统的单中心试点验证研究。
Biol Blood Marrow Transplant. 2007 Jun;13(6):715-23. doi: 10.1016/j.bbmt.2007.02.007. Epub 2007 Apr 23.
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