Abstract: The patient was a 38-year woman. Six years prior to presentation, she developed erythema and papules with occasional pruritus in both labium majora, which gradually confluenced into plaques with the formation of superficial erosion and ulcer; extension into the vulva and crissum occurred later. Half a year prior to presentation, a subcutaneous firm nodule measuring about 1.5cm in diameter with intact epidermis, developed on the right submaxilla, associated with mild swelling in the area; the nodule enlarged gradually. A subcutaneous induration measuring 0.5 cm in diameter was observed in the left chin 2 months later. She also reported a 20-year history of dry eye and dry mouth. The patient tested postive for antinuclear antibody （granular pattern, with a titer of 1 ∶ 320） and for anti-Sjogren syndrome type A （SSA） antibody. Histologically, there was a focal infiltration of lymphocytes in tissue of minor salivary glands. The pathology of lesions on the right labium majus showed a dense dermal infiltration with S100- and CD1a-positive, irregularly-shaped histiocyte-like cells with abundant eosinophilic cytoplasm and few mitotic figures. Needle biopsy of the right submaxilla area showed tumor cells. A diagnosis of Langerhans cell histiocytosis and Sjogren syndrome was made based on clinical manifestation, laboratory findings and histopathological features. Combination chemotherapy with cyclophosphamide, vincristine, corticosteroids and etoposide resulted in clinical improvement.

Key words: Sjogren's Syndrome