No abstract

No abstract

No abstract

Objective Woringer-Kolopp disease (WKD), also known as localized pagetoid reticulosis, is a rare variant of mycosis fungoides as described by the World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification system for cutaneous lymphomas. Our objective was to conduct a comprehensive review that describes and evaluates patient demographics, clinical presentation, immunohistochemical findings, management, and outcomes of WKD. Methods The databases PubMed, Embase, and Cochrane Library were searched for relevant literature. Patient demographics, imaging, treatments, and other clinical characteristics were obtained. The literature search was conducted on December 7, 2020.Studies were included if they contained primary data related to WKD. Non-pertinent studies, non-English studies, non-human studies, review articles, or studies with insufficient case information were excluded. The quality of the included studies and the risk of bias were evaluated using the Grades of Recommendation, Assessment, Development, and Evaluation Group (GRADE) criteria ( 2016), Oxford Centre for Evidence-Based Medicine: Levels of Evidence (OCEBM) (March 2009), and the Methodological Index for Non-Randomized Studies (MINORS) criteria (2003).Results A total of 84 studies with 143 patients met the inclusion criteria for this study. The most common chief complaint was an asymptomatic, slow-growing, and erythematous plaque. The average time from initial skin eruption to presentation was 97.6 months.Histologically, 128 cases of WKD displayed epidermotropism (97.7%). Various therapies, including radiotherapy, surgery, and local chemotherapy, were used. In total, 92 (78.6%) cases had complete remission and 11 (9.4%) cases recurred. Conclusions WKD is a rare cutaneous T-cell lymphoma that often presents as a single plaque on the extremities with an indolent course and a favorable prognosis. However, it is often misdiagnosed, leading to delays in treatment. The diagnosis of WKD involves clinical appraisal, a biopsy of suspicious lesions, and immunohistochemistry. Monotherapy appears to be preferred compared to combination therapy for WKD with radiotherapy demonstrating among the highest cure rates and lowest recurrence. Future retrospective and prospective studies are needed to accurately determine the epidemiology, prognosis, and effective treatments for WKD. Limitations include a possibility of missed studies, heterogeneity in reporting methods, publication, and selection bias.What is already known about this topic?• Woringer-Kolopp disease (WKD) is a rare variant of mycosis fungoides as described by the World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification system for cutaneous lymphomas.• Qualitative systematic analysis can generate new insights compared to individual findings.What does this study add?• Demonstrates the need for accurate diagnosis of Cutaneous T-Cell Lymphomas.

IGA). The Itch Numerical Rating Scale ratings had the strongest correlation with symptoms domain scores. The SDM scores in all 3 domains were significantly higher for patients who reported their disease was not well controlled. During follow-up, correlations between changes in SDM domains and changes in patientreported measures (PtGA, NRS itch, and overall control) ranged from moderate to strong (r s = 0.41-0.83). Correlations between changes in SDM domains and changes in IGA were weak (r s = 0.16-0.33). Notably, when no change was observed in IGA, PtGA, NRS itch, and overall control, the median change in SDM scoring was 0 across domains. Overall, SDM scores behaved as we expected. The weak correlation between change in IGA and change in SDM likely reflects a true discordance between patients' and physicians' assessments of disease over time.The psychometric properties of an instrument may vary depending on the type of skin disease under study. These data add to the validation base of the SDM and support its use in routine clinical practice for quickly assessing health-related QOL in patients with AD.