White : Diseases of the Skin - Collagen Vascular Diseases

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Gary M. White & Neil H. Cox

Diseases of the Skin

13	Collagen Vascular Disease

RELAPSING POLYCHONDRITIS

General aspects, diagnosis, and investigations

This is probably an autoimmune disease involving antibodies against type II collagen in cartilage. It is associated in about 25% of cases with other disorders, such as vasculitis, rheumatoid arthritis, and Behçet syndrome.

Clinical features

The external ear is most commonly affected, presenting as sore, red, swollen ears, but with sparing of the non-cartilaginous earlobes (Fig. 13.71). Concurrent fever, arthritis, nasal soreness, and red eyes are all likely, although some patients have a very low-grade inflammatory process. Vasculitic lesions and erythema nodosum-like lesions may be a feature. Involvement of trachea and heart valves are less common but potentially fatal. The ears may become chronically swollen, and nasal bridge collapse similar to that of Wegener granulomatosis may occur. Non-speciﬁc markers of inflammation such as the ESR may be grossly elevated.

Fig. 13.71 Relapsing polychondritis affecting the ear. Note the sparing of the lobe of the ear, in which there is no cartilage. Other cartilaginous tissue may be affected by this inflammatory process; in this case the patient also had chondritis of the nose, scleritis, and arthralgia.

Differential diagnosis

The most likely feature to present to a dermatologist is the ear disease, for which the main differential diagnosis is infection (chondritis or perichondritis), trauma and related disorders such as auricular pseudocyst, and occasionally contact dermatitis; the speciﬁc sparing of the earlobe would be unlikely in the latter. Wegener granulomatosis is in the differential of the nasal features, but symptoms are usually intranasal and in the province of the otorhinolaryngologist. Disorders such as polymorphic light eruption, Jessner lymphocytic inﬁltrate, and sarcoidosis may all cause redness of the pinna but do not cause the tenderness of relapsing polychondritis.

Treatment

Signiﬁcant lesions with fever and malaise are generally treated with systemic corticosteroids, and, if necessary, with other immunosuppressive agents such as azathioprine. Some cases with localized mild symptoms are controlled quite adequately with simple treatments such as NSAIDs.