Abstract
Sjögren's syndrome (SS) is a chronic inflammatory autoimmune disorder characterized by xerostomia and xerophthalmia. We report the case of a 15-year-old girl with sicca symptoms, submandibular gland swelling, and facial erythema. Biopsy of minor salivary glands revealed infiltration of lymphocytes and plasma cells. Scintigraphy of salivary glands showed submandibular and parotid gland dysfunction. Although the 1996 European classification criteria for SS were fulfilled, evidence was lacking for autoimmunity (findings were negative for rheumatoid factor, antinuclear antibody, and antibodies against SS-A and SS-B) . This suggests that the patient's sicca symptoms may have developed by mechanisms different from typical SS. The initial diagnosis of SS was made in 2001, but according to the revised European classification criteria in 2002, the diagnosis became no longer appropriate. At present, we regard this case as suggestive of SS. However, we also consider that Mikulicz disease, which is characterized by symmetrical swelling of lacrimal or salivary glands, would be a reasonable alternative diagnosis.
