The skin manifestation of acute graft-versus-host disease: clinical and pathological analysis.

We report 11 cases of acute graft-versus-host disease (GVHD); 10 occurred after bone marrow transplantation (BMT) and one following transfusion of nonirradiated whole blood in a patient with severe combined immunodeficiency (SCID). According to the Seattle Classification, 5 cases were of Grade III, 2 Grade II, and 4 Grade I. The skin rash developed between the 6th to 47th day (22 +/- 12 day) after transplantation or transfusion, and was usually manifested initially as erythematous macules or papules in the neck or upper chest. The rash resolved in scaling or became purpuric, pigmented or erythrodermic in severe cases. The grading of skin pathology correlated well with clinical severity of GVHD. Four of the five who had advanced GVHD (Grade III) died. In all 4 cases but one (the SCID case), the skin pathology showed extensive basal vacuolization and multiple dyskeratotic cells. None of the others with mild or focal basal vacuolization and few dyskeratotic cells progressed into advanced GVHD. Three of the 5 cases with advanced GVHD received an underdose of cyclosporin A. These results suggest that severe GVHD carries a poor prognosis and an adequate dose of cyclosporin A is important in alleviating the severity of GVHD and reducing the mortality. The sero-virologic study performed during the skin rash period was negative, suggesting that the skin rash following BMT is mainly caused by GVHD and always precedes intestinal or hepatic manifestations.