Systemic Lupus Erythematosus

The most obvious lesions of the head and neck that can be seen in SLE are those involving the skin. The most characteristic dermatologic feature of lupus is the malar rash (Fig. 1). Also known as the "butterfly rash," because of its shape across the cheeks and bridge of the nose, the malar rash is an erythematous and edematous eruption that classically spares the nasolabial folds. A similar rash may be seen on the forehead and chin. It may or may not result from exposure to sunlight, but is often abrupt in onset and can last for days. Malar rashes occur in 30% to 60% of patients. Discoid lupus (Fig. 2) is less frequent, occurring in 15% to 30% of patients. It too may occur in a malar distribution; however, discoid lesions can occur anywhere on the face or scalp, in the pinnae, behind the ears, or on the neck. Discoid lesions tend to be discrete plaques, often erythematous with an adherent scale that extends into hair follicles. They can progress to lesions with indurated margins of erythema and hyperpigmentation, and central atrophic scarring. Discoid lesions have been reported to result in perforation of the pinna (2). Other rashes that can appear in the head and neck area include the photosensitive subacute

FIGURE 1 Systemic lupus erythematosus: butterfly rash. Source: American College of Rheumatology.
FIGURE 2 Discoid lupus. Source: American College of Rheumatology.

cutaneous lupus, tumid lupus (3), and lupus panniculitis. Panniculitis has been reported to occur in isolation on the pinna (4) and to lead to nasal septal perforation (5). Alopecia can occur diffusely or in patches. The oral mucosa (Fig. 3) may also be affected by shallow ulcerations, most typically on the hard palate, but also on the tongue and buccal mucosa. Hyperkeratotic, lichen planus-like plaques have been reported on the buccal mucosa and palate (6). One report of laryngeal inflammation in a lupus patient was associated with cricoarytenoid arthritis (7), although cricoarytenoid arthritis occurs substantially more frequently in RA than in any other rheumatic disease. The nasal mucosa may be affected by ulcerations, as well (8), with resultant perforation (9). Autoimmune hearing loss

Lupus Arthritis
FIGURE 3 Systemic lupus erythematosus: oral ulcerations. Source: American College of Rheumatology.

(10,11) has uncommonly been reported to occur in SLE patients, as well as those with antiphospholipid antibodies. Case reports have suggested that inflammatory, autoimmune, or vasculitic mechanisms may play a role, but the precise pathophysiology involved remains obscure (12). Tinnitus may be a common symptom in lupus patients, with or without hearing loss (13). Rarely, nasal and auricular chondritis may be seen in patients with lupus (14). Spontaneous jugular vein thrombosis has very rarely been reported to occur in patients with discoid lupus and antiphospholipid antibodies (15). Central nervous system manifestations in SLE patients will occur from time to time, but a discussion of these events is beyond the scope of this chapter. It is important to note, however, that not all central nervous system events that take place in SLE patients can be due to the SLE itself; a diligent search for an infectious agent or hemorrhagic or thrombotic event should always be undertaken when an SLE patient presents with central nervous system manifestations such as seizures, severe headache, visual disturbance, or change in mood, personality, or cognition.

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