Over the years, a 64-year-old man has had repeated bouts of tenderness of the cartilaginous part of both ears that typically last for one to two weeks (see accompanying figure). The fleshy lobe portion of the ears have not been affected. The patient has not experienced shortness of breath, chest pain, dyspnea, or any eye complaints during these episodes. He had symptoms of degenerative arthritis but did not have evidence of joint effusions.
The answer is E: relapsing polychondritis, a multisystem disease that attacks cartilage and connective tissue. Cartilage contains large amounts of type II collagen, and autoanti-bodies to this antigen have been implicated in the pathogenesis of the disease. All types of cartilage may be involved including the elastic cartilage of the ears and nose, the hyaline cartilage of peripheral joints, the spinal fibrocartilage, and the cartilage in the tracheobronchial tree.1 Proteoglycanrich structures in the eye, heart, blood vessels, and inner ear may also be involved.1
The disease is characterized by episodic, widespread, and potentially destructive inflammation. Middle-aged men and women are most commonly affected, but the disease can occur at either end of the age spectrum. The most common initial symptom is acute pain, erythema, and swelling of the external ear, followed by joint pain. Fever, lethargy, and weight loss are common.
Respiratory system involvement is the most common serious manifestation of relapsing polychondritis. Cough, hoarseness, stridor, and wheezing may occur because of disintegration of the airway cartilages. Pneumonia is the most common cause of death.1
The natural history of polychondritis is unpredictable and may have an episodic, smoldering or fulminant course. Bouts of acute inflammation, healing over a few weeks, with recurrences over several years characterize the usual course. Mild episodes may be managed with nonsteroidal anti-inflammatory drugs. The majority of patients require prednisone in doses of 0.75 mg to 1 mg per kg with rapid tapering as soon as there is a clinical response. Immune suppression with methotrexate, cyclophosphamide, azathioprine, cyclosporine, or dapsone is reserved for more aggressive cases.
Cellulitis of the ear characteristically involves the entire ear, including the ear lobe, and will usually spread on the face. Bilateral, recurrent episodes would be highly uncommon.
Allergic contact dermatitis that affects the ear is often caused by nickel found in wire rim spectacles or earrings. Only the portions of the ear in direct contact with the metal would be affected.
Discoid lupus may start as an erythematous, tender plaque but will typically lead to a progressive scarring lesion instead of a condition that comes and goes.
Skin cancers, such as basal cell and squamous cell carcinomas, often affect the ear but will generally cause only localized surface change, scaling, and erosion. Like cutaneous lupus, once started, they will slowly progress and generally do not resolve without specific therapy.