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Key Clinical Message The simultaneous occurrence of pyoderma gangrenosum and systemic lupus erythematosus is exceedingly rare and poses diagnostic challenges due to the similarity of skin manifestations in both conditions. The exact relationship between the two conditions remains unclear; however, it is hypothesized that immune dysregulation and neutrophilic infiltration may play a role in the development of pyoderma gangrenosum in SLE patients. Clinicians should be vigilant in recognizing such uncommon associations to ensure prompt and appropriate management.