Published online May 16, 2022. doi: 10.12998/wjcc.v10.i14.4509
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
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.
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).
Lymphoma is a severe complication of SS, shown by the reported unique case of cutaneous MALT lymphoma with SS.
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.