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病例漫谈9:移植物抗宿主病的病例研究 [复制链接]

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Case Study: Graft-Versus-Host Disease
移植物抗宿主病的病例研究


原文链接:http://www.derm101.com/clincase/clincase.aspx?fileID=clincasestudy1_1

INITIAL PRESENTATION
首发症状



        A 38-year-old Filipino male presented with lichen planus-like plaques and dyspigmentation of the hands, lips, and oral mucosa. Eight months earlier he had received an allogeneic bone marrow transplant for the treatment of a hematologic malignancy. At the time of presentation, the patient was undergoing a slow tapering off from oral tacrolimus and prednisone. The onset of oral and cutaneous symptoms occurred de novo with no history suggestive of a preceding acute graft-versus-host reaction (i.e., gastrointestinal or hepatic dysfunction, acral erythema, erythroderma, or other erythematous eruptions).
        一名38岁的菲律宾男性,在手部、嘴唇,以及口腔黏膜出现类扁平苔藓斑块和异常色素沉着。八个月前,他接受了异基因骨髓移植,治疗恶性血液病。在症状出现的初期,患者通过口服他克莫司和强的松来缓解症状。患者虽然没有上述急性移植物抗宿主反应的病史,但却复发口腔和皮肤的发病症状。(例如:胃肠道或肝功能障碍、肢端红斑、红皮病,或者其它的红斑出疹)。

        The lichenoid form of chronic cutaneous graft-versus-host disease (GVHD) was suspected because of the morphology and pattern of the lesions in the setting of the patient's chemotherapeutic history. The lesions were most pronounced on the trunk and distal extremities and consisted of asymptomatic violaceous, hyper- and hypo-pigmented plaques (Figures 1 and 2 ). Following a typical course of chronic GVHD, in contrast with that of lichen planus, the patient's nails remained uninvolved. Furthermore, there was no evidence of sclerodermoid change. As the pattern became more widespread, the patient developed exquisitely tender, lacy-white, and hypertrophic plaques on the buccal mucosa and tongue (Figures 3 and 4 ). At the time of dermatologic consultation, the patient's oral lesions were so painful that they prevented him from eating adequate meals.
        慢性表皮移植物抗宿主病呈现苔藓样斑块,推测是由于患者的一系列化学疗法引起的病变。这些病变主要出现在躯干和四肢的远端部位,由无症状、紫罗兰色、深色或浅色斑块组成(图1和2)。典型的慢性移植物抗宿主病,与扁平苔藓相比,患者的指甲部分并不发生病变。此外,患者未见硬皮病样变化。由于病变类型越来越广泛,患者会出现触痛、白色花边纹,以及口腔黏膜和舌头上的肥厚性斑块(图3和4)。在接受皮肤科会诊时,患者由于口腔病损非常痛苦,以致进食相当困难。



Figure 1. Violaceous hyperpigmented macules on the palms.
图1手心出现紫罗兰色过度色素沉着斑点



Figure 2. Hypopigmented lesions on the lips.
图2 嘴唇部位出现白色病变





Figure 3. Erosive plaque on the buccal mucous membranes.
图3 口腔粘膜出现腐蚀斑块





Figure 4. Erosive plaque on the tongue.
图4舌头上出现腐蚀斑块



HISTOLOGY
病史


        Cutaneous punch biopsies were performed, and the results were consistent with the clinical presentation of chronic GVHD. A lichenoid pattern with pigment incontinence and lymphocyte tagging of apoptotic keratinocytes was observed.
        患者进行皮肤穿孔活检,结果与慢性移植物抗宿主病的临床表征相一致。活检观察到色素失禁的癣斑和角质细胞凋亡的淋巴细胞标记。

THERAPY
治疗


        The patient's cutaneous symptoms improved with an increase in his dose of tacrolimus alone. However, his oral mucous membrane lesions remained. Increasing immunosuppression with oral prednisone was considered. However, in an attempt to reduce the risk of opportunistic infections associated with long-term use of systemic corticosteroids, a trial of localized intraoral ultraviolet light was proposed. Biweekly intraoral PUVA treatments were initiated (Figure 5 ), and his symptoms improved rapidly. Twenty milligrams of methoxsalen (8-MOP) was given 1 hour prior to intraoral UVA using a standard dental composite curing light at an intensity of 0.5 J/cm2. The dose was cautiously increased, avoiding UV burns, to a maximum dose of 4.0 J/cm2.
        随着服用他克莫司剂量的增加,患者的皮肤症状得到改善。但是,口腔黏膜并未好转。推测是由于口服强的松导致免疫抑制逐渐增强。为了降低因长期使用系统性皮质类固醇所带来的机会性感染的风险,有人提出一项口腔内紫外光局部照射试验。患者接受两周一次的紫外光照射治疗,症状得到快速改善(图5)。患者首先服用20mg甲氧沙林,1小时之后,使用标准的牙科复合材料进行口腔内长波紫外线照射治疗,治疗光强度为0.5 J/cm2。谨慎增加光强度,避免紫外线烧伤,最大光强度为4.0 J/cm2。



Figure 5. Intraoral UVA treatment.
图5 口腔内UVA治疗



Figure 6. Only a small erosion is left after intraoral PUVA treatment.
图6 口腔内PUVA治疗后仅留存一处小区域糜烂



FOLLOW UP
随访


        After 1 month of PUVA treatments (Figure 6), the oral lesions had greatly decreased in size, and the patient's ability to tolerate opening his mouth to eat became normal. He continued UVA maintenance treatments and at nine months remained asymptomatic.
        长波紫外光治疗一个月之后(图6),患者口腔病损面积已经有了很大程度的缩小,患者能够正常进行进食。持续治疗九个月后,症状消失。



责任编辑:李锡海,王影影

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