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Relapsing polychondritis - Skin
See also in: External and Internal Eye
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Relapsing polychondritis - Skin

See also in: External and Internal Eye
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Contributors: Noah Craft MD, PhD, Lindy P. Fox MD, Lowell A. Goldsmith MD, MPH, Michael D. Tharp MD
Other Resources UpToDate PubMed

Synopsis

Relapsing polychondritis is an episodic, progressive, inflammatory autoimmune disorder involving cartilaginous structures such as the ears, nose, larynx, and tracheobronchial tree. It often affects multiple other organ systems as well. Signs and symptoms of relapsing polychondritis include auricular, nasal, costal and laryngotracheobronchial chondritis, arthropathy, ocular inflammation (eg, episcleritis), audiovestibular damage, cardiovascular disease (aortitis, aortic aneurysm or regurgitation), cutaneous vasculitis, CNS disturbances (eg, cranial neuropathies, seizures), and renal involvement. Flares are accompanied by fever, asthenia, weight loss, and sometimes liver or lymph node enlargement. Relapsing polychondritis is frequently associated with other autoimmune diseases, Behçet disease, and myelodysplastic syndromes. Complications include saddle-nose deformity/joint deformity, vertigo, tinnitus, pulmonary infection, laryngotracheobronchial stricture, scleritis or iritis, epiglottitis, blindness, valve regurgitation, aortic dissection, renal failure, and death. Relapsing polychondritis is more common in whites and the middle-aged.

Relapsing polychondritis should be distinguished from cellulitis based on its characteristic locations of predilection and constellation of associated symptoms.

Ocular manifestations of relapsing polychondritis are frequent (59% of cases) and include conjunctivitis, keratitis, scleritis, episcleritis, uveitis, retinopathy (cotton wool spots and intraretinal hemorrhages), and in those with associated vasculitis one can see optic nerve involvement and cranial nerve palsies.

Nasal pain, hoarseness, throat pain, and difficulty talking are common presenting symptoms.

Several clinical criteria have been developed for establishing the diagnosis of relapsing polychondritis (see Diagnostic Pearls).

Codes

ICD10CM:
M94.1 – Relapsing polychondritis

SNOMEDCT:
72275000 – Relapsing polychondritis

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

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Therapy

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References

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Last Updated: 10/26/2017
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Relapsing polychondritis - Skin
See also in: External and Internal Eye
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Relapsing polychondritis : Fever, Ear, Hoarseness, Nose, Painful skin lesions, Arthralgia, Dysphagia, Otalgia, Wheezing
Clinical image of Relapsing polychondritis
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