Stevens-Johnson syndrome following Sjogren's syndrome

BACKGROUND: Sicca syndrome after Stevens-Johnson syndrome is classically de scribed. However, to our knowledge, authentic Sjogren's syndrome following epidermal necrolysis has not been described previously. CASE REPORT: A 36-year-old woman with Stenven-Johnson syndrome developed tr ansient hepatitis and a persistent sicca syndrome. Fourteen years later an authentic Sjogren's syndrome was identified with presence of antinuclear an d anti-SSA antibodies and lymphocytes infiltration of the accessory salivar y glands, i.e. grade IV disease in the Chisholm classification. DISCUSSION: The initial mucosal destruction observed in our patients may ha ve laid the ground for Sjogren's syndrome via abnormal antigen presentation in a predisposed dysimmune context.