Systemic therapy for cutaneous T-cell lymphoma: who, when, what, and why?

INTRODUCTION: CTCL are rare neoplasms. Optimal care requires integrated use of diagnostic and treatment modalities spanning multiple specialties. Current instruments for patient risk stratification and disease measurement across all anatomical compartments are suboptimal. A common treatment dichotomy between early (Dermatology) and advanced stage (Hematology-Oncology) has hindered accrual of long term outcome data. Thus, important facts about natural history, such as frequency and determinants of stage progression, and the impact of specific treatment modalities on these endpoints, are not known. Areas covered: One of the most important decisions in the management of CTCL is when to start systemic therapy and what agents to use. This review provides background information to understand why systemic therapy is needed and what goals are currently achievable. Expert commentary: Risk-adapted approaches, based on better knowledge of host and tumor biology, and more accurate disease measurement tools are needed to optimize the use of specific systemic therapies.

Xanthomatized Neutrophilic Dermatosis in a Patient With Myelodysplastic Syndrome.

We present an original case report of a 58-year-old man with a history of histiocytoid Sweet and myelodysplastic syndrome who presented with a new onset of tender plaques on his oral commissures. A biopsy revealed a dense dermal neutrophilic infiltrate, leukocytoclasis without frank vasculitis, and, most notably, the presence of many xanthomatized cells. Clinical presentation and histologic features were most consistent with an acute neutrophilic dermatosis, which we believe to be a xanthomized variant of Sweet syndrome. Recent cases have described patients with similar clinical features and neutrophilic dermatosis in the setting of a normolipemic xanthomatosis. These cases were ultimately diagnosed as neutrophilic xanthoma, suggesting a possible histologic overlap between neutrophilic xanthoma and Sweet syndrome. Given these findings, we propose xanthomatized Sweet syndrome as a new histologic variant.

A Retrospective Analysis Comparing the New Standardized Letter of Recommendation in Dermatology with the Classic Narrative Letter of Recommendation.

Background: In an effort to avoid numerous problems associated with narrative letters of recommendation, a dermatology standardized letter of recommendation was utilized in the 2014-2015 resident application cycle. Objective: A comparison of the standardized letter of recommendation and narrative letters of recommendation from a single institution and application cycle to determine if the standardized letter of recommendation met its original goals of efficiency, applicant stratification, and validity. Methods: Eight dermatologists assessed all standardized letters of recommendation/narrative letters of recommendation pairs received during the 2014-2015 application cycle. Five readers repeated the analysis two months later. Each letter of recommendation was evaluated based on a seven question survey. Letter analysis and survey completion for each letter was timed. Results: Compared to the narrative letters of recommendation, the standardized letter of recommendation is easier to interpret (p<0.0001), has less exaggeration of applicants' positive traits (p<0.001), and has higher inter-rater and intrarater reliability for determining applicant traits including personality, reliability, work-ethic, and global score. Standardized letters of recommendation are also faster to interpret (p<0.0001) and provide more information about the writer's background or writer-applicant relationship than narrative letters of recommendation (p<0.001). Limitations: This study was completed at a single institution. Conclusions: The standardized letter of recommendation appears to be meeting its initial goals of 1) efficiency, 2) applicant stratification, and 3) validity. (J Clin Aesthet Dermatol. 2016;9(9):36-2.).

Sézary Syndrome: Clinical and Biological Aspects.

Sézary syndrome (SS) is a rare and aggressive type of cutaneous T cell lymphoma (CTCL) characterized by an intensely pruritic, exfoliative rash, known as erythroderma, with cutaneous and systemic dissemination of clonal CD4+ T cells into the blood and lymph nodes. This review aims to present recent advancements in the biological and clinical aspects of SS. We begin by providing an overview of the diagnostic criteria for SS and reviewing some of its epidemiological and clinical aspects. We then discuss updates in the etiology of this elusive disease and the genetic and molecular landscapes that define it. Finally, we provide a short overview of the current therapeutic strategies for SS as well as recent advances in the prognosis of this disease. A brief set of recommendations is provided regarding future directions in research and therapy.