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[Stevens-Johnson syndrome followed by Gougerot-Sjögren syndrome]
C de Roux Serratrice1, J Serratrice, B Granel
1Service de Médecine interne, CHU Timone, 265, boulevard Jean Moulin, F 13385 Marseille. pdisdier@ap-hm.fr
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.