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Relapsing polychondritis - External and Internal Eye
See also in: Overview,Cellulitis DDx
Other Resources UpToDate PubMed

Relapsing polychondritis - External and Internal Eye

See also in: Overview,Cellulitis DDx
Contributors: Rajini Murthy MD, Aditi Jani MD, Paritosh Prasad MD, Susan Burgin MD
Other Resources UpToDate PubMed


Relapsing polychondritis is an autoimmune multisystemic condition affecting cartilaginous structures such as the ears, nose, eyes, joints, larynx, and tracheobronchial tree.

The course and clinical aspects of relapsing polychondritis vary significantly across patients, and early manifestations of disease are often extremely subtle and go unrecognized. 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; central nervous system (CNS) disturbances (eg, cranial neuropathies, seizures); and renal involvement.

Auricular chondritis is the most common clinical feature, and nasal chondritis is present in around 25% of patients at diagnosis. Nasal pain, hoarseness, throat pain, and difficulty talking are common presenting symptoms.

Ocular manifestations of relapsing polychondritis are frequent (59% of cases) and can affect almost any part of the eye, which makes diagnosis based on ocular findings alone extremely difficult. These findings include conjunctivitis, keratitis, scleritis, episcleritis, uveitis, retinopathy (cotton wool spots and intraretinal hemorrhages), and in those with associated vasculitis, optic nerve involvement and cranial nerve palsies.

Flares are accompanied by fever, asthenia, weight loss, and sometimes liver or lymph node enlargement.

Relapsing polychondritis is frequently associated with other autoimmune connective tissue disorders, vasculitis, 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 the White population and between the ages of 40 and 60 years. The disease equally affects male and female patients.


M94.1 – Relapsing polychondritis

72275000 – Relapsing polychondritis

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Differential Diagnosis & Pitfalls

  • Cellulitis / infectious Perichondritis
  • Pseudocyst of auricle
  • Chondrodermatitis nodularis helicis
  • Allergic contact dermatitis
  • Seborrheic dermatitis
  • Atopic dermatitis
  • Granuloma annulare
  • Granulomatosis with polyangiitis
  • Trauma
  • Rheumatoid arthritis
  • Early congenital syphilis (saddle-nose deformity)
  • Polyarteritis nodosa
  • Sarcoidosis
  • Drug-induced photosensitive reaction
  • VEXAS syndrome

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Last Reviewed:07/26/2021
Last Updated:08/04/2021
Copyright © 2024 VisualDx®. All rights reserved.
Relapsing polychondritis - External and Internal Eye
See also in: Overview,Cellulitis DDx
A medical illustration showing key findings of Relapsing polychondritis : Fever, Ear, Erythema, Hoarseness, Nose, Painful skin lesions, Arthralgia, Dysphagia, Otalgia, Wheezing
Clinical image of Relapsing polychondritis - imageId=4580899. Click to open in gallery.  caption: 'Diffuse pink erythema of the ear, sparing the ear lobule.'
Diffuse pink erythema of the ear, sparing the ear lobule.
Copyright © 2024 VisualDx®. All rights reserved.