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Isolated Neurofibroma of Lid Margin in a Case of Paediatric Sjogren’s Syndrome - A Rare Case Report

Somya Dulani, Sachin Diagavane, Seema Lele, Rakesh Juneja, Anand Tibedawal and Netra Adalakiya

Sjögren syndrome (SS) is a chronic, inflammatory and autoimmune disease characterized by progressive lymphocytic and plasma cell infiltration of the salivary and lacrimal glands. It is observed in 90% of women in the age group 35-45 yr. The primary SS is very rare in childhood. It may be a primary or secondary disorder to other autoimmune disorders, such as systemic lupus erythematosus (SLE), rheumatoid arthritis, scleroderma and biliary cirrhosis; but association with neurofibromatosis is not reported till date. Isolated neurofibroma of lid is very rare, though it can be associated with Von Recklinghausen’s disease. We report here a case of Primary Pediatric Sjogren’s Syndrome presented with isolated neurofibroma of lid margin.