Abstract

Blau Syndrome (BS) is a rare autoinflammatory granulomatous disorder which mostly develops at an early age (less than four years) and is described by granulomatous dermatitis, symmetric arthritis and recurrent uveitis. This paper presented a case of a 5-yearold female child with joint pain and swelling of four-year duration. Initially, her diagnosis was made as juvenile idiopathic arthritispolyarticular Rheumatoid Factor (RF) negative and was initiated on methotrexate along with bridge steroids (prednisolone). When she presented to us, she had skin coloured micropapular rashes mostly non follicular over body, from last nine months and after skin and synovial biopsy, the diagnosis was reviewed as early onset sarcoidosis/BS- sporadic type. This rare report highlights that BS is an under recognised and reported childhood arthritis which may have multisystem involvement. The onset of arthritis without ocular or skin manifestations may be mistaken as juvenile idiopathic arthritis.

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