Spontaneous regression of primary cutaneous diffuse large B‐cell lymphoma, leg type: A case series and review of the literature

Author:

Winkler Manuel1ORCID,Albrecht Jana Dorothea123,Sauer Christian4,Kordaß Theresa4,Guenova Emmanuella5ORCID,Livingstone Elisabeth6,Wobser Marion7,Mitteldorf Christina8,Géraud Cyrill1910,Nicolay Jan Peter123

Affiliation:

1. Department of Dermatology, Venereology, and Allergology, University Medical Center and Medical Faculty Mannheim Heidelberg University Mannheim Germany

2. Clinical Cooperation Unit Dermato‐Oncology German Cancer Research Center Heidelberg Germany

3. Section of Clinical and Experimental Dermatology, Medical Faculty Mannheim Heidelberg University Mannheim Germany

4. Institute of Pathology, University Medical Center and Medical Faculty Mannheim Heidelberg University Mannheim Germany

5. Department of Dermatology, Lausanne University Hospital, Faculty of Biology and Medicine University of Lausanne Lausanne Switzerland

6. Department of Dermatology, Venereology, and Allergology University Hospital Essen Essen Germany

7. Department of Dermatology, Venereology, and Allergology University Hospital Würzburg Würzburg Germany

8. Department of Dermatology, Venereology and Allergology University Medical Center Göttingen Göttingen Germany

9. Section of Clinical and Molecular Dermatology, Medical Faculty Mannheim Heidelberg University Mannheim Germany

10. European Center for Angioscience, Medical Faculty Mannheim Heidelberg University Mannheim Germany

Abstract

AbstractPrimary cutaneous diffuse large B‐cell lymphoma, leg type (PCDLBCL, LT) is a subtype of cutaneous B‐cell lymphoma with unfavorable prognosis usually requiring aggressive polychemotherapy for disease control. Only single cases of spontaneous regression of PCDLBCL, LT are reported in the literature, peaking 3 months post‐biopsy following a clinical history of no longer than 1 year. Here, we report the first case of a spontaneously relapsing and remitting PCDLBCL, LT with complete regression after a clinical history of more than 9 years and thus an atypically indolent clinical course. The female patient presented with recurrent erythematous, non‐ulcerated, non‐raised plaques of the right lower leg for 6 years. Pathological workup and exclusion of a systemic disease confirmed the diagnosis of PCDLBCL, LT. Due to the history of repeated spontaneous remission, no therapy was initiated. Nine years after first occurrence the patient presented with complete clinical remission lasting for 64 months. We retrospectively identified four additional PCDLBCL, LT patients with spontaneous remission lasting up to 53 months. Our data provide evidence for a distinct PCDLBCL, LT patient subgroup that clinicians should be aware of and warrants a watch‐and‐wait treatment regime.

Publisher

Wiley

Reference15 articles.

1. The 2018 update of the WHO-EORTC classification for primary cutaneous lymphomas

2. Cutaneous B‐cell lymphomas—pathogenesis, diagnostic workup, and therapy;Nicolay JP;J Dtsch Dermatol Ges,2016

3. Identification of Somatic Mutations in Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type by Massive Parallel Sequencing

4. Spontaneous regression of primary diffuse large B‐cell lymphoma, leg type;Alcántara‐González J;Actas Dermosifiliogr,2014

5. Spontaneous regression of a primary cutaneous diffuse large B-cell lymphoma, leg type

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