Woringer‐Koloppdisease (localized pagetoid reticulosis): a systematic review

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.

An Indolent Forearm Plaque: A Quiz

“…Pagetoid reticulosis, also known as Woringer–Kolopp disease, is a rare form of cutaneous T-cell lymphoma that presents as a single, slow-growing, hyperkeratotic or scaly plaque usually on the extremities ( 1 ). It is classified by the European Organisation for Research and Treatment of Cancer System as a variant of mycosis fungoides ( 2 ).…”

“…While the clinical and histopathological results are relatively constant, the immunophenotypic profile is more heterogeneous: T cells are most often CD4 – /CD8 + , but cases with CD4 + /CD8 – , CD4 – /CD8 – , or CD4 + /CD8 + phenotype have also been documented. The disease is inconsistently CD30 + ( 1 ). No additional imaging or testing is recommended, as no cases of distant or lymph node metastasis have been described in the localized form of pagetoid reticulosis ( 1 ).…”

“…The disease is inconsistently CD30 + ( 1 ). No additional imaging or testing is recommended, as no cases of distant or lymph node metastasis have been described in the localized form of pagetoid reticulosis ( 1 ). Radiotherapy or wide excision should be considered initially, as the cure rate is excellent and the recurrence rate is very low ( 1 , 5 ).…”

“…No additional imaging or testing is recommended, as no cases of distant or lymph node metastasis have been described in the localized form of pagetoid reticulosis ( 1 ). Radiotherapy or wide excision should be considered initially, as the cure rate is excellent and the recurrence rate is very low ( 1 , 5 ). Combined therapies and other modalities such as topical corticosteroids ( 1 ), photodynamic therapy ( 6 ), local chemotherapy (topical alkylating agents, interferon alpha-2b ( 7 ), imiquimod ( 8 )), or alitretinoin ( 9 ) may be used.…”

“…Radiotherapy or wide excision should be considered initially, as the cure rate is excellent and the recurrence rate is very low ( 1 , 5 ). Combined therapies and other modalities such as topical corticosteroids ( 1 ), photodynamic therapy ( 6 ), local chemotherapy (topical alkylating agents, interferon alpha-2b ( 7 ), imiquimod ( 8 )), or alitretinoin ( 9 ) may be used. In a systematic review that included 143 patients, 92 patients had complete remission after treatment (most of them with radiotherapy, surgery, or topical corticosteroids) and 11 cases recurred with a median time to recurrence of 38.5 months.…”