Nail SCC is found nearly exclusively in adults and predominantly in men. There are multiple effective treatment possibilities including Mohs surgery, distal digital amputation, and early evidence suggesting radiotherapy.
Background
Cutaneous T-cell lymphoma (CTCL) is a group of lymphoproliferative disorders that includes mycosis fungoides (MF) and Sezary syndrome (SS). T-Plastin (PLS3) is an actin-bundling protein that has been found to be highly expressed in Sezary cells but not in normal PBMCs. Here, we describe the value of using PLS3 as a sensitive molecular marker for differentiating stages of MF from SS, and for monitoring development of SS from MF.
Objectives
To determine the relationship between PLS3 expression level and SS, and disease progression and response to treatment in patients with MF/SS.
Methods
Total RNA from PBMCs from normal volunteers, MF/SS, and psoriasis patients were measured by quantitative PCR for PLS3 gene expression.
Results
In PBMCs from MF/SS and psoriasis patients, PLS3 expression was increased markedly in SS (greater than 400-fold) compared to that seen in early and late stage MF patients without SS cells or in patients with psoriasis. In a patient whose disease progressed to SS from MF, the PLS3 level in PBMCs showed an increased with disease progression. With treatment, the level of PLS3 decreased with chemotherapy and bone marrow transplantation.
Conclusions
PLS3 expression is a valuable and sensitive molecular biomarker to differentiate MF from SS. The measure of this gene may have value in conjunction with gene rearrangement studies to monitor disease severity or progression from MF to SS. Furthermore, PLS3 is not expressed in other inflammatory skin diseases, and may be valuable to distinguish SS from other cutaneous diseases associated with generalized erythroderma.
The most common cancer in both men and women is basal cell carcinoma (BCC). Although most primary and recurrent BCCs have high cure rates with standard therapies, advanced BCCs present a greater treatment challenge, especially in cosmetically and functionally sensitive areas. In patients unable to undergo surgery or radiation therapy, hedgehog inhibitors can be used neoadjuvantly to reduce tumor size, decreasing the extent and complexity of any subsequent surgery and providing either a cure or palliation. The goal of this review is to summarize the pharmacology, efficacy, and safety of systemic hedgehog inhibitors, as well as their role in daily practice as neoadjuvant therapy. Relevant English-language literature was identified and evaluated based on results from database searches of PubMed. Terms searched included, but were not limited to, "vismodegib," "Erivedge," "sonidegib," "DE225," "BCC," and "neoadjuvant treatment." Additional literature was identified from the reference lists of previously identified articles. The authors' personal experience in treating advanced BCC using hedgehog inhibitors has been incorporated into the recommendations made herein.
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