Background: Sicca syndrome after Stevens-Johnson syndrome is classically described. However, to our knowledge, authentic Sjögren's syndrome following epidermal necrolysis has not been described previously.
Case report: A 36-year-old woman with Steven-Johnson syndrome developed transient hepatitis and a persistent sicca syndrome. Fourteen years later an authentic Sjögren's syndrome was identified with presence of antinuclear and anti-SSA antibodies and lymphocytes infiltration of the accessory salivary glands, i.e. grade IV disease in the Chisholm classification.
Discussion: The initial mucosal destruction observed in our patients may have laid the ground for Sjögren's syndrome via abnormal antigen presentation in a predisposed dysimmune context.