Dermatoarthritis, familial histiocytic M14.3

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Arthritis; Arthritis familial granulomatous; familial granulomatous; familial histiocytic dermatoarthritis

History
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Zayid and Farraj, 1973

Definition
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So far very rarely described, familial disease manifesting in early childhood, characterized by granulomatous cutaneous and subcutaneous papules, preferably on the backs of the hands and feet as well as the extensor sides of the extremities. In addition, mutating arthritis of the peripheral joints, especially of the hands and feet, occurs. Typical is eye involvement with uveitis, glaucoma or cataract. Relationships to multicentric reticulohistiocytosis (Weber and Freudenthal) are discussed.

Etiopathogenesis
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Autosomal dominant inherited.

Manifestation
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First decade of life.

Localization
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Joints, back of hands and feet, face, extremity extensor sides.

Clinical features
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  • Morning stiffness and painful bilateral swelling of the hand, metacarpophalangeal and proximal interphalangeal, elbow, knee and ankle joints. Increasing (seronegative) deforming rheumatoid polyarthritis, especially in the hands with pronounced restriction of movement. Additionally painful swellings on the backs of the hands and feet and in the area of the ankles (synovial cysts). Intermittent generalised papulo-nodular livid-brownish exanthema (usually coupled with iritis) on the face, ears and extensor sides of the extremities, with preference for the backs of the hands and feet.
  • Plate-like subcutaneous indurations, glaucoma, uveitis, iritis, cataract with early visual impairment and blindness.
  • Radiological skeletal changes: Pronounced joint deformities and signs of severe periarticular bone resorption.

Laboratory
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Rheumatoid factor neg., ACE levels normal.

Histology
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Diffuse, dense, tumor-like infiltrate of mononuclear and rarely duplex histiocytic cells (in other authors also multinuclear giant cells) and abundant endothelial proliferated capillaries. In older nodules and subcutaneous plaques mainly thickened hyaline collagen bundles and fibroblasts, but few histiocytes.

Differential diagnosis
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Multicentric reticulohistiocytosis; juvenile rheumatoid arthritis; sarcoidosis; dermochondrocorneal dystrophy (François syndrome).

Therapy
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Arthritis and iritis only temporarily morbostatically influenced with low-dose glucocorticoids; no response to NSAIDs.

Literature
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  1. Blue EB (1985) Familial granulomatous arthritis, iritis, and rash. J Pediatr 107: 689-693
  2. Jabs, DA et al (1985) Familial granulomatous synovitis, uveitis, and cranial neuropathies. On J med 78: 801-804
  3. Parenti A, Cipriani R et al (1987) Familial Histiocytic Dermatoarthritis. At J Dermatopathol 9: 491-496
  4. Pastores GM, Michels VV, Stickler GB et al (1990) Autosomal dominant granulomatous arthritis, uveitis, skin rash, and synovial cysts. J Pediatr 117: 403-408
  5. Rosenbaum JT et al (2003) With a mere nod, uveitis enters a new era. Am J Ophthalmol 136: 729-732
  6. Zayid I, Farraj S (1973) Familial histiocytic dermatoarthritis. A new syndrome. On J Med 54: 793-800

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020