Case Report
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. May 16, 2022; 10(14): 4509-4518
Published online May 16, 2022. doi: 10.12998/wjcc.v10.i14.4509
Cutaneous mucosa-associated lymphoid tissue lymphoma complicating Sjögren's syndrome: A case report and review of literature
Ying Liu, Jian Zhu, Yan-Hong Huang, Qian-Ru Zhang, Li-Ling Zhao, Ruo-Han Yu
Ying Liu, Yan-Hong Huang, Qian-Ru Zhang, Li-Ling Zhao, Ruo-Han Yu, Department of Rheumatology, Beijing Tsinghua Changgung Hospital, School of Clinical Medicine, Tsinghua University, Beijing 102218, China
Jian Zhu, Department of Rheumatology and Immunology, The First Medical Center, Chinese PLA General Hospital, Beijing 100853, China
Author contributions: Liu Y performed all the literature review, clinical analysis, and wrote the manuscript with contributions from all authors; Zhu J and Huang YH designed and supervised the study; Zhu J, Zhang QR, Zhao LL and Yu RH revised the manuscript.
Informed consent statement: Written informed consent was obtained from the patient for publication of this report and all accompanying images.
Conflict-of-interest statement: The authors declare that there is no conflict of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Jian Zhu, MD, Professor, Department of Rheumatology and Immunology, The First Medical Center, Chinese PLA General Hospital, No. 28 Fuxing Road, Haidian District, Beijing 100853, China. jian_jzhu@126.com
Received: July 16, 2021
Peer-review started: July 16, 2021
First decision: October 18, 2021
Revised: October 30, 2021
Accepted: March 25, 2022
Article in press: March 25, 2022
Published online: May 16, 2022
Processing time: 300 Days and 23.7 Hours
Abstract
BACKGROUND

The association of Sjögren's syndrome (SS) and lymphoma is similar. Mucosa-associated lymphoid tissue (MALT) or extranodal marginal zone B-cell lymphoma was the most common lymphomatous histology in SS patients. MALT in SS patients is frequently located in the parotid gland, while MALT lymphoma of the skin with SS is an exceedingly rare entity that needs to be recognized.

CASE SUMMARY

A 60-year-old woman presented with a 3-year history of progressive dry mouth associated with a 1-year history of enlarging cutaneous nodules. Physical examination revealed two hard subcutaneous nodules on her right lower leg. The results of Schirmer’s test were positive, despite the absence of dry eyes. Labial salivary gland biopsy revealed lymphocytic infiltration and chronic inflammation with a focus score of 2. The patient was diagnosed with SS. She underwent resection of one cutaneous nodule, and histopathological analysis identified the nodule as MALT lymphoma. Her dry mouth symptoms improved, and the nodules decreased after 6 mo of treatment with hydroxychloroquine sulfate and chemotherapy (thalidomide, cyclophosphamide, and dexamethasone).

CONCLUSION

Lymphoma is a severe complication of SS, shown by the reported unique case of cutaneous MALT lymphoma with SS.

Keywords: Sjögren's syndrome; Mucosa-associated lymphoid tissue; Lymphoma; Skin; Nodule; Case report

Core Tip: Lymphoma is a severe complication of Sjögren's syndrome (SS) and mucosa-associated lymphoid tissue (MALT) lymphoma is the most common type. We report a rare case of cutaneous MALT lymphoma with SS. A literature review was performed to provide information on the condition’s clinical manifestations and associated extensive sites. Our case highlights that the skin is rarely involved aside from the parotid gland, orbital adnexa, lung, thyroid, and stomach. Patients may have no symptoms; thus, regular physical assessments are required.