Jornal Vascular Brasileiro
https://jvascbras.org/article/doi/10.1590/1677-5449.210003
Jornal Vascular Brasileiro
Case Report

Atypical presentation of Sjogren’s syndrome with MALT lymphoma mimicking chronic venous ulcers

Apresentação atípica da síndrome de Sjogren com linfoma MALT que mimetiza úlceras crônicas venosas

Marta Wasilewska

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Abstract

Abstract: Chronic ulcerations of the lower extremities are quite a common condition amongst adults, most often caused by chronic venous insufficiency. Irrespective of the main underlying cause, chronic limb ulcerations are usually associated with significant symptoms, impairing daily functioning. Improper or delayed diagnosis and inadequate treatment increase the risk of serious complications, including limb amputations. Malignancies can develop secondary to chronic leg ulcers. About 2.4% of ulcers arising from chronic venous stasis undergo malignant transformation. Squamous cell carcinoma is the most common type of malignancy found in chronic leg ulceration biopsies. Basal cell carcinoma, sarcoma, and melanoma have all been documented infrequently. In the case described here, we found lymphoma of the marginal zone of mucosa-associated lymphoid tissue (MALT), which is an extremely rare cutaneous neoplasm of the lower extremities, but one that may have an association with autoimmune diseases.

Keywords

leg ulcer, venous insufficiency, skin neoplasms, Sjogren’s syndrome, lymphoma

Resumo

Resumo: Úlceras crônicas dos membros inferiores são uma condição bastante comum entre adultos, na maioria das vezes causadas por insuficiência venosa crônica. Independente da sua causa principal, úlceras crônicas dos membros estão geralmente associadas a sintomas significativos, prejudicando o funcionamento diário. O diagnóstico inadequado ou tardio e o tratamento inadequado aumentam o risco de complicações graves, inclusive o risco de amputação do membro. Malignidades podem se desenvolver secundariamente em úlceras crônicas da perna. Em torno de 2,4% das úlceras decorrentes de estase venosa crônica sofrem transformação maligna. O carcinoma de células escomosas é o tipo de malignidade mais comum que pode ser encontrada em biópsias de úlceras crônicas da perna. Carcinoma basocelular, sarcoma e melanoma foram raramente documentados. No caso aqui descrito, encontramos linfoma da zona marginal do tecido linfoide associado à mucosa (mucosa-associated lymphoid tissue, MALT), o qual é uma neoplasia cutânea dos membros inferiores extremamente rara mas que pode estar associada a doenças autoimunes.
 

Palavras-chave

úlcera da perna, insuficiência venosa, neoplasias cutâneas, síndrome de Sjogren, linfoma

References

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Submitted date:
01/21/2021

Accepted date:
04/28/2021

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