Clinical and Pathological Characteristics and Outcomes Among Patients With Subcutaneous Panniculitis-like T-Cell Lymphoma and Related Adipotropic Lymphoproliferative Disorders.

Importance: There is a knowledge gap about subcutaneous panniculitis-like T-cell lymphoma (SPTCL) owing to its rarity and diagnostic difficulty, resulting in an absence of well-documented large case series published to date. Objective: To generate consensus knowledge by a joint multi-institutional review of SPTCL and related conditions. Design, Setting, and Participants: This retrospective clinical and pathological review included cases initially diagnosed as SPTCL at 6 large US academic centers. All cases were reviewed by a group of pathologists, dermatologists, and oncologists with expertise in cutaneous lymphomas. Through a process of group consensus applying defined clinical and pathological diagnostic criteria, the cohort was classified as (1) SPTCL or (2) adipotropic lymphoproliferative disorder (ALPD) for similar cases with incomplete histopathological criteria for SPTCL designation. Exposures: Cases of SPTCL diagnosed between 1998 and 2018. Main Outcomes and Measures: The main outcome was disease presentation and evolution, including response to therapy, disease progression, and development of hemophagocytic lymphohistiocytosis. Results: The cohort of 95 patients (median [range] age, 38 [2-81] years; female-to-male ratio, 2.7) included 75 cases of SPTCL and 20 cases of ALPD. The clinical presentation was similar for both groups with multiple (61 of 72 [85%]) or single (11 of 72 [15%]) tender nodules mostly involving extremities, occasionally resulting in lipoatrophy. Hemophagocytic lymphohistiocytosis (HLH) was only observed in SPTCL cases. With a mean follow-up of 56 months, 60 of 90 patients (67%) achieved complete remission with a median (range) of 3 (1-7) cumulative therapies. Relapse was common. None of the patients died of disease progression or HLH. Two patients with ALPD eventually progressed to SPTCL without associated systemic symptoms or HLH. Conclusions and Relevance: In this case series of patients initially diagnosed as having SPTCL, results showed no evidence of systemic tumoral progression beyond the adipose tissue. The SPTCL experience in this study confirmed an indolent course and favorable response to a variety of treatments ranging from immune modulation to chemotherapy followed by hematopoietic stem cell transplantation. Morbidity was primarily associated with HLH.

Association of Flame-Retardant Clothing With Mycosis Fungoides: A Retrospective Analysis.

Introduction: Mycosis fungoides (MF), the most prevalent form of cutaneous T-cell lymphoma (CTCL), has been associated with a variety of environmental and occupational exposures. Flame-retardant clothing (FRC), in contrast to flame-resistant clothing, is chemically treated and may constitute a previously unrecognized occupational hazard. Objectives: To report an association between FRC and MF. Methods: After encountering several young male patients whose onset of MF coincided with the occupational use of FRC and occupation as fire fighters, we did a retrospective search. Additional biopsy proven MF patients with use of FRC were identified by the EPIC electronic medical record using the search terms "CTCL, mycosis fungoides, flame, and flame-retardant." Results: Eight MF patients, all males, ranging in age from 31 years to 64 years (median age, 35 years) with exposure to FRC were identified. MF remission was noted in three patients who discontinued FRC use and in one patient who used a cotton undershirt barrier, while disease persistence was noted in one patient who continued to use FRC. Conclusions: FRC appears to be associated with development of MF through chronic antigen stimulation. Use of FRC is an occupational hazard for fire fighters. Any patient whose MF coincides with use of FRC should avoid further exposure through avoidance or switching to clothing made from inherently flame-resistant fibers.

Vulvar Primary Cutaneous CD8+ Aggressive Epidermotropic Cytotoxic T-Cell Lymphoma.

Cutaneous T-cell lymphomas may present with a clinical course that is incongruent with the associated histologic findings. Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma classically presents as an abrupt eruption of disseminated ulcerated annular plaques with aggressive behavior and a poor prognosis. Herein we describe a vulvar primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma with a locally aggressive clinical course that was strikingly responsive to radiation therapy. As aggressive therapy involving systemic chemotherapy is indicated for primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma, appropriate clinico-pathologic correlation is crucial for preventing potentially excessive or insufficient therapeutic intervention. Our case also highlights the pivotal role of both radiation therapy and infection control in the management of aggressive cutaneous vulvar lymphomas.

Unusual presentation of Kaposi sarcoma in an HIV-negative woman.

Kaposi sarcoma typically presents as violaceous macules and papules in immunocompromised, specifically HIV-positive, patients. Its distinct clinical features often facilitate rapid diagnosis. In this article, we report a case of Kaposi sarcoma presenting as a concerning yet nondescript lesion in an HIV-negative woman. Although Kaposi sarcoma is frequently part of the differential diagnosis for skin lesions affecting HIV-positive patients, it is less frequently considered in HIV-negative individuals. Additionally, this case differs from the classic clinical presentation of Kaposi sarcoma by resembling a squamous cell carcinoma or superficial basal cell carcinoma. Therefore, it illustrates the importance of suspicious lesion biopsies to ensure accurate diagnosis and appropriate treatment.

Complete Resolution of Primary Cutaneous Anaplastic Large Cell Lymphoma With Topical Imiquimod

Primary cutaneous anaplastic large cell lymphoma (pc-ALCL) is a CD30+ subtype of cutaneous T-cell lymphoma. It typically has a very favorable prognosis; however, traditional treatment can be expensive, invasive, and associated with significant adverse events. Imiquimod is a topical toll-like receptor approved by the Food and Drug Administration (FDA) for genital warts, actinic keratosis, and primary superficial basal cell carcinoma. In previous case reports, imiquimod has been shown to be effective against pc-ALCL. We present a case of complete resolution of pc-ALCL within 8 weeks with topical imiquimod and review the current literature. J Drugs Dermatol. 2019;18(5):460-462.

Diagnosis of cutaneous T-cell lymphoma by insurance type before and after the Affordable Care Act: a national database study.

The Affordable Care Act (ACT) was implemented to increase health care access and reduce the uninsured in the age group between pediatric and Medicare populations (18-64). The association of the ACA with insurance type upon diagnosis (uninsured, Medicaid, non-Medicaid) has been investigated for otolaryngologic, gynecologic, and the top five non-skin malignancies. Such studies for cutaneous malignancies are lacking. We conducted a retrospective analysis of the prospective National Cancer Institute's Surveillance, Epidemiology, and End Results (SEER) cancer database to assess the impact of the ACA on new diagnoses of cutaneous T-cell lymphoma (CTCL) by insurance type. Unlike prior studies of other malignancies, we did not observe significant differences between rate of diagnosis of CTCL by insurance type before and after full implementation of the ACA in all states, expansion states, and non-expansion states. Skin cancers do not have screening guidelines and CTCL is an uncommon malignancy, both of which may contribute to these findings. However, Medicaid-expansion states were much closer to reducing the percentage of newly diagnosed uninsured patients with CTCL than non-expansion states. As such, it may be prudent to investigate intrinsic socioeconomic barriers to care in Medicaid patients to improve their access to care to decrease the uninsured population and improve outcomes.