Pyoderma gangrenosum as the first clinical manifestation of primary Sjögren disease: A case report and review of literature

Introduction: Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis characterized by painful cutaneous ulcers. It can appear as primary or idiopathic illness or secondary to a systemic disease. Only few cases in the literature reported the association between PG and Sjögren disease, which manifests primarily with sicca symptoms. This paper describes the remarkable initial clinical presentation of primary Sjögren disease revealed by diffuse and severe PG that improved dramatically after treatment initiation. History: A 25-year-old female, with previous history of ocular dryness, was hospitalized after the rapid progression of severe skin eruption which first started at the lower limbs, then affected her face and trunk. The transformation of the cutaneous rash from non-pruritic papules into extremely painful, well defined, hemorrhagic bullae with central scarring was clinically consistent with PG. Pathergy sign was present. Skin biopsy showed an aspect of neutrophilic dermatosis. ANA profile revealed positive anti-SSA/Ro and positive anti-SSB/La. Since the patient also had xerophthalmia, xerostomia and highly positive Schirmer’s test, she was diagnosed with PG secondary to Sjögren’s syndrome. She responded well to systemic steroids tapered to the oral form then oral cyclosporine along with local wound care. Conclusion: This is the first case report that illustrates severe PG as the first clinical manifestation of Sjögren’s syndrome, which has to be ruled out in the setting of each neutrophilic dermatosis, and can be successfully treated with corticosteroids and cyclosporine. Correspondence to: Alexandre Malek MD, Infectious diseases, at Saint Joseph University, Faculty Of Medicine/Hotel Dieu De France Hospital, Beyrouth, Lebanon, E-mail: [email protected]