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A Novel Form of Chronic Cutaneous Graft-vs-Host Disease and Its Response to Psoralen–UV-A Therapy

Daniel Creamer, MD; Claire L. Martyn-Simmons, MRCP(England); Genevieve Osborne, MRCP(England); Michelle Kenyon, MSc; Jon R. Salisbury, MD; Stephen Devereux, MD; Antonio Pagliuca, MD; Aloysius Y. Ho, MD; Ghulam J. Mufti, MD; Anthony W. P. du Vivier, MD

Arch Dermatol. 2007;143(9):1157-1162.

Background  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.

Observations  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.

Conclusions  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.

Author Affiliations: Departments of Dermatology (Drs Creamer, Martyn- Simmons, Osborne, and du Vivier) and Histopathology (Dr Salisbury), King's College Hospital; and Department of Haematological Medicine, King's College Hospital and King's College London (Ms Kenyon and Drs Devereux, Pagliuca, Ho, and Mufti), London, England.