A Rare Manifestation Of Primary Sjogren’s Syndrome With Pyoderma Gangrenosum : Case Report

Abstract

Objective: Sjögren’s Syndrome (SjS) is a systemic autoimmune disease typically presenting with sicca symptoms. However, a limited number of case reports in the literature have described rare initial presentations with pyoderma gangrenosum (PG). This report aims to underscore this infrequent association between PG and SjS by presenting another illustrative case.

Case: This case report elucidates the diagnosis and treatment of primary Sjögren’s syndrome (pSS), focusing on the ulcerated lesion that manifested on the leg of a 54-year-old female patient with no underlying health conditions. The findings are discussed in the context of the existing literature.

Discussion: SjS is a multisystemic autoimmune disease characterized by lymphocyte infiltration and damage to tissues, especially exocrine glands. Although sicca symptoms are typically the initial clinical findings, PG can rarely manifest as the primary symptom, presenting as an ulcerative skin lesion predominantly located on the lower extremities. There is currently no standard treatment recommendation in the existing guidelines for this rare condition.

Conclusion: The co-occurrence of PG and SjS is uncommon, yet it may serve as a warning for associated systemic diseases, such as SjS. Treatment may involve a combination of corticosteroids and disease-modifying antirheumatic drugs (DMARDs).