Creamer Daniel, Martyn-Simmons Claire L, Osborne Genevieve, Kenyon Michelle, Salisbury Jon R, Devereux Stephen, Pagliuca Antonio, Ho Aloysius Y, Mufti Ghulam J, du Vivier Anthony W P
Department of Dermatology, King's College Hospital, Denmark Hill, London SE5 9RS, England.
Arch Dermatol. 2007 Sep;143(9):1157-62. doi: 10.1001/archderm.143.9.1157.
Chronic cutaneous graft-vs-host disease (GVHD) is generally classified by whether lesions have a lichenoid or sclerodermatous morphology. Other unusual clinical forms have been reported that exhibit the features of dermatomyositis and lupus erythematosus. Within a large population of individuals who underwent allogeneic stem cell transplantation because of hematologic malignancy, a group of patients was identified in whom severe and persistent eczema developed.
We prospectively evaluated 10 adult patients with unexplained eczematous dermatosis after allogeneic hematopoietic stem cell transplantation. The dermatosis developed between 2 and 18 months (mean, 7.5 months) after receipt of the transplant, exhibited the typical clinical features of dermatitis, and became erythrodermic in each case. The patient group had strong risk factors for chronic cutaneous GVHD: 8 had received a transplant from an unrelated donor, 7 had evidence of extracutaneous GVHD, and 7 had a history of acute cutaneous GVHD. Sampling of lesional skin revealed the histologic features of GVHD coexisting with the changes of dermatitis. The patients were treated with topical corticosteroid and systemic immunosuppressive agents. Six patients also received psoralen-UV-A. Four patients achieved prolonged remission. Six patients died, 5 of infective complications and 1 of relapsed leukemia.
The eczematous dermatosis observed represents a novel form of chronic cutaneous GVHD that we named eczematoid GVHD. Eczematoid GVHD is an aggressive, chronic dermatosis that requires substantial immunosuppression therapy to achieve control. It is associated with a poor prognosis. Although atopy can be transmitted to an individual from a hematopoietic stem cell transplant, none of the donors in this series gave a history of an atopic disorder. Therefore, other factors must be implicated in provoking the expression of an eczematous phenotype in individuals with underlying chronic graft-vs-host activity.
慢性皮肤移植物抗宿主病（GVHD）通常根据皮损是否具有苔藓样或硬皮病样形态进行分类。还报道了其他表现为皮肌炎和红斑狼疮特征的不寻常临床形式。在因血液系统恶性肿瘤接受异基因干细胞移植的大量个体中，发现了一组出现严重且持续性湿疹的患者。
我们前瞻性评估了10例异基因造血干细胞移植后出现不明原因湿疹性皮炎的成年患者。这种皮肤病在接受移植后2至18个月（平均7.5个月）出现，表现出皮炎的典型临床特征，且每例均发展为红皮病。该患者组有慢性皮肤GVHD的强烈危险因素：8例接受了来自无关供体的移植，7例有皮肤外GVHD的证据，7例有急性皮肤GVHD病史。对皮损皮肤进行采样显示GVHD的组织学特征与皮炎改变并存。患者接受了外用糖皮质激素和全身免疫抑制剂治疗。6例患者还接受了补骨脂素 - 紫外线A治疗。4例患者实现了长期缓解。6例患者死亡，5例死于感染并发症，1例死于白血病复发。
观察到的湿疹性皮炎代表了一种新的慢性皮肤GVHD形式，我们将其命名为湿疹样GVHD。湿疹样GVHD是一种侵袭性慢性皮肤病，需要大量免疫抑制治疗才能得到控制。它与预后不良有关。虽然特应性可以通过造血干细胞移植传递给个体，但本系列中的供体均无特应性疾病史。因此，一定有其他因素参与引发具有潜在慢性移植物抗宿主活性个体中湿疹样表型的表达。
