Faculty Bibliography

Cutaneous T cell lymphoma (CTCL) represents a heterogeneous group of extranodal non-Hodgkin lymphomas in which monoclonal T lymphocytes infiltrate the skin. The mechanism of CTCL development is not fully understood, but likely involves dysregulation of various genes and signaling pathways. A variety of treatment modalities are available, and although they can induce remission in most patients, the disease may recur after treatment cessation. Owing to relatively low incidence and significant chronicity of disease, and the high morbidity of some therapeutic regimens, further clinical trials are warranted to better define the ideal treatment option for each subtype of CTCL.

Lymphomatoid papulosis is often regarded as a low-grade variant of cutaneous T cell lymphoma (CTCL). Given the excellent long-term prognosis, recent consensus guidelines indicate that patients can be monitored off therapy. We report a case of a 67-year-old man who presented with lymphomatoid papulosis, with necrotic papules that have been intermittently present for over forty years.

Cutaneous T cell lymphoma is a heterogeneous group of lymphomas characterized by the accumulation of malignant T cells in the skin. The molecular and cellular etiology of this malignancy remains enigmatic and what role antigenic stimulation plays in the initiation and/or progression of the disease remains to be elucidated. Deep sequencing of the tumor genome revealed a highly heterogeneous landscape of genetic perturbations and transcriptome analysis of transformed T cells further highlighted the heterogeneity of this disease. Nonetheless, using data harvested from high-throughput transcriptional profiling allowed us to develop a reliable signature of this malignancy. Focusing on a key cytokine signaling pathway, previously implicated in CTCL pathogenesis, JAK/STAT signaling, we used conditional gene targeting to develop a fully penetrant small animal model of this disease that recapitulates many key features of mycosis fungoides, a common variant of CTCL. Using this mouse model, we demonstrate that T cell receptor engagement is critical for malignant transformation of the T lymphocytes and that progression of the disease is dependent on microbiota.

The United States (US) Department of Defense(DoD) has been a leader in using telecommunicationstechnology to provide remote medical care. The DoDhas been using telemedicine for more than twentyyears to provide medical services to military personneldeployed throughout the world, and has largelyinfluenced the development of teledermatology. Theexperiences of early military teledermatology serviceshave yielded valuable lessons that have been essentialto the creation of successful civilian programs.

We report an HIV-negative, 55-year-old manwith recurrent Kaposi's sarcoma (KS) of the lowerextremities, who does not fit into any of thefour previously described variants of KS: classicKS, AIDS-related KS, iatrogenic KS, and AfricanendemicKS. There are reports in the literature ofchildhood-onset KS, which is thought to be dueto an inherited immune deficiency that confers ahigher susceptibility to human Herpesvirus-8 (HHV-8), which is the virus that is known to cause KS. Ourpatient may be affected with an inherited immunedeficiency that has predisposed him to KS, and thismutation also may account for his prostate andbladder cancer.