Epstein Barr virus‐associated lymphoproliferative‐disorders primarily involving the skin

In cases of solid organ or bone marrow transplantation, up to 2 to 10% of patients may develop lymphoproliferative disorders (LPD), often induced by Epstein‐Barr virus (EBV). Despite a morphology mimicking malignant lymphoma, in some cases the lesions will disappear completely after the degree of immunosuppression is lowered. Lately, similar processes have been described in non‐transplant, immunosuppressed patients. A SNOMED search was performed on the database of three hospitals between 1990 and 1997, to identify patients with immunosuppression‐relaied lymphoproliferative disorders (IR‐EPD) involving primarily the skin. Two patients were identified. One was 2 years after kidney transplantation, and the other was being treated with methotrexate for dermatomyositis. In both biopsies, there was a diffuse perivascular proliferation of large lymphocytes with ample cytoplasm and pleomorphic nuclei, associated with extensive dermal and subcutaneous necrosis. Immunohistochemical studies revealed expression of CD20, CD45RO, CD43, CD30, EBV‐LMP1, and EBV‐NA2 by the atypical lymphocytes in both cases and, in one case, of the EBV‐transcriptional replication activation protein. In both cases the lesions completely disappeared and have not recurred. Primary involvement of the skin by IR‐LPD is very rare. Based on our results, it is possible that some of these cases in the skin contain EBV and co‐express CD30 and T‐ and B‐ccll markers. The diagnosis of IR‐LPD should be considered in cutaneous lymphoid proliferations in immunosuppressed patients. Belore rendering an unequivocal diagnosis of malignant lymphoma, reduction of immunosuppression and follow‐up of 4‐8 weeks should be considered.