A clonal cutaneous CD30+lymphoproliferative eruption in a patient with evidence of past exposure to hepatitis E

The patient was a 52-year-old white man who had worked in remote areas of t he world during the past 2 years, including an extended period in rural are as of Central Africa and in Central and South America. He had no acute illn esses during the 2-year period except for rare, mild, upper respiratory tra ct infections. For approximately 1 year, however, he had developed recurren t, papular-vesicular, slightly painful lesions on the fingers and palms, th at spontaneously healed over weeks to months (Fig. 1). The patient had no o ther concurrent illnesses and no abnormal laboratory findings, except for p ositive enzyme-linked immunoabsorbent assay (ELISA) for immunoglobulin G (I gG) antibodies for hepatitis E virus (HEV) using a recombinant expressed HE V antigen (Genelabs Technologies, Inc., San Antonio). Prolonged treatment w ith minocycline did not appear to moderate the lesions. At approximately 2. 5 years after the development of his first cutaneous lesion, however, the p atient reported that he had had no new lesions for over 3 months. A biopsy specimen showed an intraepidermal vesicle with prominent epidermal necrosis and reticular degeneration (Fig. 2). Within the epidermis, there was a dense infiltrate of lymphoid cells. The majority of these cells were pleomorphic with prominent nucleoli and frequent mitotic figures (Fig. 3). Sheets of atypical cells were found in the subjacent dermis. The infiltrate extended down into the reticular dermis. With extension into the dermis, t he infiltrate became more polymorphous with more small lymphoid cells, larg e numbers of eosinophils, and some plasma cells located more deeply. Immunohistochemical stains for CD3 (DAKO), CD4 (Becton Dickinson), CD8 (Bec ton Dickinson), CD15 (LeuM1, Becton Dickinson), CD20 (L-26, DAKO), CD30 (Be r-H2, DAKO), CD45RO (UCHL1, DAKO), S-100 protein (DAKO), T-cell intracellul ar antigen (TIA) (Coulter), epithelial membrane antigen (EMA) (DAKO), KP-1 (CD68, DAKO), MAC-387 (DAKO), Epstein-Barr virus (EBV) latent membrane anti gen-1 (LMP-1, DAKO), and EBV-encoded nuclear antigen 2 (EBNA2, DAKO) were p erformed on formalin-fixed tissue using the ABC method with DABA as the chr omagen. CD3 showed diffuse membrane staining of the large atypical lymphoid cells, as well as the majority of the small lymphoid cells (Fig. 4). CD4 showed po sitive membrane staining of the large atypical lymphoid cells and the major ity of the small lymphoid cells. CD8 showed only scattered light membrane s taining of small lymphoid cells. CD15 was negative, and CD20 showed foci of groups of small lymphoid cells mainly within the reticular dermis. CD30 sh owed positive membrane and paranuclear staining of the targe atypical cells , most abundant within the epidermis and papillary dermis (Fig. 5). CD45RO showed positive membrane staining of the large atypical cells and the major ity of the small lymphoid cells. S-100 protein showed increased dendritic c ells within the surrounding viable epidermis and the subjacent papillary de rmis (Fig. 6). TIA showed granular staining in the large atypical lymphoid cells and only rare staining in small lymphoid cells (Fig. 7). EMA staining was essentially negative. KP-1 showed only scattered positive cells mainly in the lower papillary and the reticular dermis. MAC-387 showed membrane s taining in the viable epidermis (Fig. 8). LMP-1 and EBNA2 for EBV were nega tive within the lymphoid cells as well as within the overlying epidermis. Gene rearrangement studies showed a beta-T-cell receptor gene rearrangement . The monoclonal band was detected with VJ1, VJ2, and D1J2 primer sets. The T-cell receptor beta rearrangement assay has a sensitivity of 61% and a sp ecificity of 94% for the detection of a monoclonal rearrangement in T-cell lymphomas for which amplifiable DNA can be recovered. Electron microscopy was performed on formalin-fixed material, positive-fixe d with 2.5% phosphate-buffered glutaraldehyde and further with 1% osmium te troxide by standard techniques. Intracellular, 50-60-nm, cytoplasmic, spher ical, viral-like particles were identified (Fig. 9).