Cases reported "Polychondritis, Relapsing"

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1/4. A case of relapsing polychondritis involving the tragal and the conchal bowl areas with sparing of the helix and the antihelix.

    We describe a 65-year-old white man with a 21-year history of recurrent, afebrile episodes of painful, tragal, conchal bowl and eyelid swelling accompanied by occasional conjunctivitis. The remainder of the auricle was not involved. Episodes were both self-remitting and responsive to intramuscular steroid injections. Cutaneous and cartilaginous tissues were examined histologically following a therapeutic debulking procedure. The histologic features included dermal edema, vascular dilatation, and small vessel inflammation with a dense polymorphous inflammatory infiltrate rich in eosinophils. Perichondrial inflammation and cartilage degeneration with fibrosis were characteristically observed. Bacterial cultures demonstrated normal flora. This case fulfills the revised diagnostic criteria of relapsing polychondritis. It demonstrates an unusual presentation within the disease spectrum of relapsing polychondritis with tragal and conchal bowl involvement and sparing of the helix and the antihelix.
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keywords = vessel
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2/4. Relapsing polychondritis: a course over 20 years with cerebral involvement.

    Relapsing polychondritis is a chronic inflammatory disease associated with an autoimmune disorder in cartilaginous tissue, eyes, labyrinth, blood vessels, and central nervous system. We describe a 75-year-old woman who presented with a 20-year history of dyspnea, inspiratory stridor, and polyarthritis. She developed dysmorphism of both ears and a saddle nose approximately 10 years earlier. Subsequently, she suffered from hearing loss and a tremor. A T2-weighted magnetic resonance imaging scan of the brain revealed multiple, spotted signal intensities. Immunohistochemical analysis of a serum sample showed antibodies to cartilaginous tissue, which were further identified on immunoblotting as antibodies to type II collagen. The extremely prolonged course of disease (>20 years) until a correct diagnosis was made is remarkable. Also, cerebral involvement, which was most likely caused by cerebral angiitis, and which, to our knowledge, has never previously been reported in this form, was detected. Arch Otolaryngol head neck Surg. 2000;126:1495-1498
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keywords = vessel
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3/4. Exudative retinal detachment in relapsing polychondritis : case report and literature review.

    OBJECTIVE: To report the atypical ocular symptoms (arterialized conjunctival vessels, exudative retinal detachment) that can be the presenting manifestations of relapsing polychondritis. DESIGN: Observational case report and literature review. methods: A complete ocular and systemic evaluation was performed on a patient with relapsing polychondritis and exudative retinal detachment. MAIN OUTCOME MEASURES: Retinal, choroidal, and scleral findings. RESULTS: A 73-year-old man with relapsing polychondritis presented with a unilateral large bullous exudative retinal detachment and marked choroidal and scleral thickening bilaterally. CONCLUSIONS: Ophthalmologists should consider relapsing polychondritis in the differential diagnosis of exudative retinal detachment. A combination of echographic and laboratory findings can assist in the accurate diagnosis of this rare condition.
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ranking = 1
keywords = vessel
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4/4. Large vessel arteritis in relapsing polychondritis.

    A healthy 58-year-old woman presented with recurrent swelling and pain of the nose and both auricules. Bruits were heard over both carotid arteries. magnetic resonance angiography revealed stenosis of both internal carotid arteries. Relapsing polychondritis was diagnosed. These symptoms improved after treatment with prednisolone and azathioprine. Although relapsing polychondritis is sometimes associated with systemic vasculitis, large vessel arteritis is rare and can negatively affect prognosis. We conclude that the detection of systemic vascular lesions, including those involving the central nervous system, can play an important role in the diagnosis of relapsing polychondritis and that early treatment is essential for a good outcome.
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