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Synonym: Familial granulomatous synovitis-uveitis and cranial neuropathies. A rare chronic inflammatory condition characterized by familial granulomatous synovitis, arthritis, dermatitis and uveitis, and cranial neuropathies.

Onset in childhood. Granulomatous synovitis; symmetric, boggy polysynovitis of the hands and wrists, resulting in boutonniere deformities. Recurrent nongranulomatous acute iridocyclitis. Cranial neuropathies: bilateral neurosensory loss; 6th cranial nerve palsy. Inheritance is autosomal dominant.

Bibliography

D. A. Jabs, J. L. Honk, W. B. Bias, E. C. Arnett:
•   Familial granulomatous synovitis, uveitis and cranial neuropathies.
    American Journal of Medicine, New York, 1985, 78: 801-804.

E. B. Blaus:
•   Familial granulomatous arthritis, iritis and rash.
    Journal of Pediatrics, St. Louis, 1985, 107: 689-693.

Sergio I. Magalini, Sabina C. Magalini, Giovanni de Francisi:
•   Dictionary of Medical Syndromes.
    J. B. Lippincott Company, Philadelphia. 1990.

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