Primary Sjögren syndrome: report of a 10 years old girl with local edema and positivity of anti SS-A and anti SS-B autoantibodies

Abstract

Sjögren-Larsson syndrome (SLS) is an autoimmune disease, uncommon in childhood. We report a case of SLS in a 10-year-old girl with a history of tumor, calor and rubor in the back of her toes almost every month, which resolved in 4-5 days without therapy. She did not complain of dry mouth or dry eyes. The laboratory fi ndings showed high infl ammation markers, rheumatoid factor 128 IU, Waaler-Rose 256 IU, anti nuclear antibody (ANA) 1/640, SSA (anti Sjögren antigen A) and SSB (anti Sjögren antigen B) positive and hypergammaglobulinemia. The Schirmer’s test resulted to be pathologic, the ultrasonography images and biopsy of minor salivary glands revealed focal periductal lymphocytic infi ltrate and sialoduct ectasia class IV of juvenile Sjögren syndrome. The juvenile Sjögren syndrome is frequently under-diagnosed. Clinical manifestations in children might be different from the adult form, although laboratory fi ndings may be similar to those found in adults.

Keywords

• Sjögren syndrome, anti Sjögren antigen, anti SS-A, anti SS-B.