BACKGROUND: Sjögren’s syndrome is a chronic, systemic disorder of an autoimmune nature, and its primary etiopathogenetic events are not known. Previous studies have found elevated incidence of malignancies in patients with primary Sjögren’s syndrome. However, there are few reports regarding the association of Sjögren’s syndrome with skin cancers, especially with multiple skin cancers developed within a short time.

CASE REPORT: We reported an unusual case of a patient with primary Sjögren’s syndrome who suffered from rapidly developed facial and neck skin cancers within two years.

CONCLUSIONS: Sjögren’s syndrome associated with skin cancer is rare. Our case report suggests that Sjögren’s syndrome patients require continuous follow-up with conventional cancer examination, including skin biopsy for suspected skin lesions.

Keywords: Sjogren's Syndrome, Skin Neoplasms, Autoimmune Diseases