Extracorporeal photochemotherapy is a new form of immunotherapy which involves the extracorporeal photoinactivation of peripheral blood cells by 8-methoxypsoralen in the presence of ultraviolet A irradiation, followed by readministration of the cells. To explore the efficacy of this therapy in the treatment of autoimmune disease, four patients with a lengthy history of corticosteroid and immunosuppressive drug-resistant pemphigus vulgaris were initiated on extracorporeal photochemotherapy. Three patients experienced a complete remission in cutaneous disease expression, permitting discontinuation of medications in two and a substantial decrease in the third. Significant reductions in serum antiepidermal cell antibody titers occurred in all four patients. The treatments were well tolerated without the occurrence of adverse events. These results in a small number of patients suggest that extracorporeal photochemotherapy may prove to be a useful tool in the treatment of aggressive autoimmune disease.
One newly recognized form of T-cell lymphoma is breast implant-associated anaplastic large cell lymphoma (biALCL), which appears in close proximity to breast implants. The number of reported cases of biALCL is increasing and warrants careful attention by clinicians to more effectively diagnose and treat affected individuals. As pertinent to dermatologists, the objective of this paper is to present the associated cutaneous features of this clinical entity along with the pathogenesis, management, and clinical outcomes. biALCL is a T-cell lymphoma in which malignant T-cells are characterized by large pleomorphic and anaplastic morphology and immunoreactivity for CD30, similar to primary cutaneous anaplastic large cell lymphomas (pcALCL). It has a favorable clinical outcome like nonimplant-associated pcALCL and involves the fibrous capsule around the implant, which creates an immunologically privileged site with a peri-implant effusion (seroma). More rare presentations are of a solitary mass. Appropriate management of biALCL is the complete surgical removal of the implant and total capsulectomy. Dermatologists should be aware of the occurrence of this entity in patients who have breast implants because patients may present specifically for breast-related cutaneous findings or have incidental cutaneous changes noted during a skin examination. The recognition and timely diagnosis of biALCL is critical to prevent progression to more advanced disease, ensure adequate treatment with removal of the implant, and avoid unnecessary aggressive systemic chemotherapy.
To accurately assess tumor burden in cutaneous T-cell lymphoma as well as to determine the number of residual normal circulating T cells, it is necessary to accurately distinguish malignant cells. Because cutaneous T-cell lymphoma cells regularly display many normal phenotypic markers of T cells (CD2+, CD3+, CD4+) these surface proteins have been of limited value. We have used a set of monoclonal antibodies with specificity for those T-cell receptor proteins containing variable regions on the beta chain to distinguish normal from malignant T cells in patients with cutaneous T-cell lymphoma.The results revealed an unanticipated and profound expansion of the malignant cell populations (59% to 87% of blood T cells) in six patients with total T-cell counts in the normal or near normal range. By subtracting the percentages of malignant T cells, identified in this manner, from the total T-cell counts, we found that the residual normal T-cell compartments were small (0 to 0.155 x 10(9)/L) in four of the six patients. Sézary cell counts by peripheral blood smear analysis by routine light microscopy underestimated the number of malignant T cells. Markedly elevated CD4/CD8 ratios (10 to 90) occurred in all cases, reflecting expansion of the CD4+ malignant population and parallel reduction of the normal residual CD8+ subset.Patients with erythrodermic cutaneous T-cell lymphoma often have markedly depressed levels of normal blood T cells, to the range seen in advanced acquired immunodeficiency syndrome, and absolute numbers of malignant cells substantially exceed those recognized with less sensitive techniques.
