Severe Adverse Skin Reaction Used For Sclerodermatous Chronic Graft Versus Host Disease Of The Skin

Treatment of sclerodermatous chronic graft versus host disease remains a daunting challenge. Supportive care measures remain the primary mode of therapy with little evidence of treatments that reverse the process. Photopheresis has been used with varying success in this disorder. Recently, case reports and case series have reported on the successful use of imatinib in this disorder(1, 2) Drug intolerance was reported in four of the patients treated.

We report a case of a 40 year old man with extensive scelerodermatous chronic graft versus host disease (GVHD) of the skin ( Skin : score of 3 using the NIH Criteria).

Four years earlier, he underwent a matched unrelated stem cell transplant for chronic myelogenous leukemia. His transplant course was complicated with TTP associated with all standard immune suppressive agents (cyclosporine, tacrolimus, sirolimus). This gentleman developed progressive sclerodermatous skin GVHD. Photopheresis was carried out for two years with stabilization, but no improvement in his symptoms. Imatinib was started due to persistent symptoms (joint restriction Score of 2 in NIH criteria, > 50% of skin involvement, joint Ð score of 3 NIH). ³

Imatinib was started for progressive symptoms and within five days, he was hospitalized with a generalized erythroderma with associated skin sloughing. The Imatinib was stopped and he received high dose steroids with slow improvement of his symptoms. Skin biopsy at that time revealed subcorneal pustular dermatosis; there was no herpes viral effects and special stains were negative for bacterial or fungal organisms. There was a diffuse infiltration of neutrophils. Histological differential diagnosis included pustular psoariasis and Sneedon-Wilkinson syndrome. In view of the history, the concluding diagnosis was exanthematous pustulosis secondary to imatinib.

We recommend that close attention be paid to patients with chronic GVHD treated with imatinib and early intervention with stopping the Imatinib. Steroid worked in this case and should be considered.

• Blood 2009 Mar. Magro et al

• Arch Dermatol 2008 Sept; 144(9): 1106-9

National Institutes of Health Consensus Development Project on Criteria for Clinical Trials in Chronic Graft-versus-Host Disease: I. Diagnosis and Staging Working Group Repor Alexandra H. Filipovich,1 Daniel Weisdorf,2 Steven Pavletic, et al Biology of Blood and Marrow Transplantation 11:945-955 (2005).