[Blau syndrome or familial form of sarcoidosis with onset during infancy].

INTRODUCTION

Blau syndrome is a granulomatous disease with dominant autosomal transmission. Skin, joint and ocular manifestations usually appear in childhood.

CASE REPORTS

A father and his son had granulomatous disease with skin and joint manifestations beginning in childhood. Both patients had inflammatory polyarticular deformations of the small and medium sized joints with formation of synovial cysts. Skin manifestations were seen only in the son who presented diffuse micropapulous eruptions. Histology examination of the superficial and deep derma revealed an epithelioid granuloma without necrosis.

DISCUSSION

Our case are similar to the syndrome described by Blau who recognized the familial nature of early onset sarcoidosis and probable autosomal dominant transmission together with joint deformation and development of synovial cysts without pulmonary involvement.