Cutaneous Manifestations of Primary Sjögren's Syndrome: Pyoderma Gangrenosum as a Rare Initial Presentation - A Case Report and Literature Review.

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis that can occur as an idiopathic condition or in association with various systemic diseases. The association with Sjögren's syndrome (SS) is extremely rare, with only a few cases reported in the literature where PG developed in patients with established SS. We report the unique case of a 39-year-old female who presented with painful hemorrhagic bullae affecting the lower extremities, upper lip, and back, which rapidly evolved from nonpruritic papules. The patient also complained of arthralgia in small joints of both hands and had a history of dry mouth and eyes. Laboratory investigations revealed elevated inflammatory markers, positive antinuclear antibodies, and strongly positive anti-SSA/Ro and anti-SSB/La antibodies. Histopathological examination confirmed neutrophilic dermatosis compatible with PG. Schirmer's test was markedly positive, and salivary gland biopsy showed minimal inflammation, confirming the diagnosis of SS according to established classification criteria. The patient was initially treated with high-dose intravenous methylprednisolone and colchicine with good initial response. The patient showed excellent response with significant improvement in skin lesions and control of SS symptoms at a 3-month follow-up. This case highlights the importance of screening for underlying autoimmune connective tissue diseases in patients presenting with neutrophilic dermatoses.