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  Request for families with JRA-like autosomal dominant disease (PAPA)

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Author Topic:   Request for families with JRA-like autosomal dominant disease (PAPA)
posted 06-12-2000 10:09 PM     Click Here to See the Profile for cwise   Click Here to Email cwise     Edit/Delete Message Reply w/Quote
We are seeking rare families with disease resembling early onset, severe JRA. Clinical profile includes history of recurrent joint swelling and cystic skin lesions of early onset. Arthritis is characteristically intermittent and migratory and leads to the accumulation of sterile pyogenic material but without granuloma formation within the joint space.Disease is remarkable for strong family history, presenting in an autosomal dominant inheritance pattern. In some cases severe cystic acne, typically of pre-adolescent onset, is a component. Laboratory tests are typically normal during flares except for anemia and elevated aldolase. This disorder may be related to, or the same as, PAPA syndrome.

Carol A. Wise,PhD
TX Scottish Rite Hospital for Children
Dallas, TX


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