Primary and isolated cutaneous lymphomatoid granulomatosis following heart-lung transplantation.

Background. Lymphomatoid granulomatosis is an Epstein-Barr virus-associated B-cell lymphoproliferative disease. It is angiocentric and angiodestructiv e and involves the lungs, central nervous system and skin. Exclusive cutane ous involvement is rare and may be associated with a better outcome. Contra rily to the extra-cutaneous forms of lymphomatoid granulomatosis, it is dif ficult or impossible to detect Epstein-Barr virus DNA sequences in primary and isolated cutaneous lymphomatoid granulomatosis. Case report. A 54-year-old woman developed erythemato-violaceous lesions on both legs 3 years after a heart-lung transplantation. The diagnosis of ery thema multiforme and of drug-induced vasculitis were first: made. Because o f fever and of the rapid extension of the lesions, the patient was hospital ized. The histologic examination of the first lesions showed a perivascular infiltrate, without epidermotropism, composed of histiocytes, lymphocytes and plasma cells. Immunohistochemistry revealed the presence of a predomina ntly T-cell infiltrate with some large B cells. Subsequent biopsies were di agnosed as high grade B-cell lymphoma. Polymerase chain reaction analysis a s well as in situ hybridation study showed the presence of Epstein-Barr vir us load in the lesions. There was however no serologic evidence of viral re activation. Extensive systemic evaluation revealed no visceral or bone marr ow involvement. Despite antiviral treatment and CHOP polychemotherapy, the patient died 3 months after her admission. Discussion. This observation of lymphomatoid granulomatosis is particular b ecause of its exclusive cutaneous involvement associated with a fulminant e volution to high grade B lymphoma, The presence of a context of iatrogenic immunosuppression underlies the role of altered immune cellular functions i n the initiation and/or progression of lymphomatoid granulomatosis and stre ngthens the role of a viral agent in its pathogenesis. We suggest that the presence of Epstein-Barr virus, which is generally not associated with the isolated cutaneous forms of lymphomatoid granulomatosis, may have played a role in this fulminant evolution to high grade B lymphoma.