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Familial histiocytic dermatoarthritis syndrome 4


Familial Histiocytic Dermatoarthritis Syndrome 727f59h 727f59h 727f59h 727f59h 4

General: Autosomal dominant; manifestations in childhood or adolescence; progressive.

Ocular: Glaucoma; bilateral uveitis; complicated cataract.

Clinical: Multiple histiocytic cutaneous nodules (face, ears, upper and lower extremities); subcutaneous plaques apparent on palpation and thickened, lichenified skin; arthropathy with symmetrical destructive arthritis mainly of hands and wrists but also observed on feet and elbows; possible hearing loss; cardiac and skeletal muscle failure; severe synovitis with arthritis mutilans.

Campbell DA, Edwards NL. Multicentric reticulohistiocytosis: systemic macrophage disorder, Baillieres Clin Rheumatol 1991; 5:301-319.

Geeraets WJ. Ocular Syndromes, 3rd ed. Philadelphia: Lea & Febiger, 1976.

Sauden Y. Diagnosis of peripheral arthropathies. Radiol Clin Biol 1974; 43:283.

Zayid J, Farraj S. Familial histiocytic dermatoarthritis: a new syndrome. Am J Med 1973; 54:793.




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