Malignant melanoma and basal cell carcinoma in a combined tumour
Authors: None De Almeida Barbosa jr, None Sales Guimarães, None De Lourdes Lopes, None Sadigursky, None Bittencourt
Journal: British Journal of Dermatology (1999)

Epstein-Barr virus-associated lymphoid hyperplasia of the eyelid characterized by intramuscular infiltrationSIR, Latent Epstein-Barr virus (EBV) infections have been implicated in various malignant conditions, including Burkitt's lymphoma in Africa, nasopharyngeal carcinoma, posttransplantation lymphoproliferative disorders, Hodgkin's lymphoma, non-Hodgkin's lymphoma in patients with AIDS, chronic active EBV infection, gastric cancer, pyothoraxassociated pleural lymphoma and smooth-muscle tumours. 1,2 We have previously reported the characteristic clinicopathological features of EBV-associated lymphoproliferative disorders: (i) subcutaneous lymphoma associated with haemophagocytosis; (ii) hydroa vacciniforme-like vesiculopapular eruptions; (iii) angiocentric lymphoma; and (iv) histiocytoid lymphoma with systemic haemophagocytic syndrome. 3 We report a further subgroup of two patients characterized by facial and eyelid swelling with infiltration of T or natural killer (NK)/T cells associated with a latent EBV infection.Patient 1.A 40-year-old woman gave a 1-year history of swollen erythema on her right eyelid.Her family history and past history were unremarkable.The eruption was localized to the eyelid, and was associated with lymphoedema (Fig. 1a).The initial differential diagnoses included dermatomyositis, lupus erythematosus profundus, lymphoid hyperplasia of ocular adnexae and granulomatous disorders such as sarcoidosis and Melkersson-Rosenthal syndrome.No abnormalities were noted in the peripheral blood cells, blood chemistry, urinalysis, immunoglobulin levels or antibodies to nuclear antigens and human T-cell lymphotropic virus type 1. Computed tomography (CT) revealed swelling of the right eyelid and masseter muscle.Antibody titre tests against EBV revealed an anti-VCA IgG level of 1 : 320, an anti-EA IgG level of 1 : 80 and an anti-EBNA level of 1 : 10.NK cell activity and lymphokine-activated killer cell activity were normal, and her HLA typing was A26(10), A24(9), B62(15), B51(5), Cw4.A biopsy of the eyelid lesion revealed dilated lymphatic vessels, pronounced lymphoedema throughout the dermis, and infiltration of small and medium-sized lymphoid cells around the vessels and in the muscles.Immunostaining demonstrated that the infiltrating cells were mainly T cells with a CD3⑀þ, CD8-, CD56-, HLA-DRþ phenotype.The eyelid swelling was promptly resolved by topical and systemic steroid therapy, but recurred soon after discontinuation of this treatment.Four years after the onset of the illness, indurated cutaneous nodules occurred on the extremities, and were associated with a fever and an elevated level of lactate dehydrogenase.A biopsy demonstrated the presence of dense infiltration by medium-sized lymphoid cells into the deep dermis and subcutaneous tissues.The tumour cells exhibited a CD3⑀þ, CD45ROþ phenotype, and a rearrangement of the T-cell receptor (TCR) gene was noted on Southern blotting analysis.In situ hybridization demonstrated that the primary eyelid lesion contained EBV-encoded small nuclear RNA (EBER)-positive cells comprising 25-40% of the infiltrating cells.In the tumorous lesions on the leg, > 80% of the infiltrating cells were positive for EBER.The EBER-positive cells did not express latent membrane protein-1.Polymerase chain reaction (PCR) analysis demonstrated the presence of the BamHI W region of the EBV gene in the skin lesions.No involvement was noted in the lymph nodes, internal organs or bone marrow.Although combination chemotherapy of cyclophosphamide, adriamycin, vincristine and prednisolone led to partial remission, new subcutaneous nodules occurred without any visceral or