Acute cutaneous lupus erythematosus

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Acute cutaneous lupus erythematosus (ACLE) usually occurs in association with systemic manifestations preceding by weeks or months the onset of a multisystem disease (Watanabe and Tsuchida,

1995; Wysenbeek et al., 1992; Yung and Oakley, 2000). Sun exposure is a common exogenous factor to be capable of precipitating ACLE (Kuhn et al., 2001a; Wysenbeek et al., 1989), and some patients even report an exacerbation of their systemic symptoms after exposure to sunlight. Furthermore, infections, especially with subtle types of viruses, or certain drugs, e.g. hydralazine, is-oniazide, and procainamide, have also been found to induce or aggravate this disease (Pramatarov, 1998; Rubin, 1999).

There are localized and generalized manifestations of ACLE (Costner et al., 2003; Fabbri et al., 2003). The localized form commonly presents as the classic "malar rash'' or ''butterfly rash'' on the central portion of the face and may only affect the skin transiently. Therefore, at the onset of disease, the patient may mistake this rash for sunburn. It usually begins with small, discrete erythematous macules and papules, occasionally associated with fine scales and gradually becomes confluent and hyperkeratotic. Facial swelling may be severe in some patients with ACLE; however, it mostly disappears without scarring and pigmentation (Norden et al., 1993; Yell et al., 1996). Similar lesions have also been found to occur on the forehead, the V-area of the neck, the upper limbs, and the trunk. In addition, patients with ACLE may have diffused thinning or a receding frontal hairline with broken hair (lupus hair), and may further present with teleangiectasias and erythema of the proximal nail fold and cuticular abnormalities (Patel and Werth, 2002). Superficial ulcerations of the oral and/or nasal mucosa are also frequently accompanied with this subtype and may cause extreme discomfort in some patients.

The generalized form of ACLE is a less common variety and may be located anywhere on the body although the preferred sites are above the waistline (Fig. 1) (Costner et al., 2003; Yell et al., 1996). The onset of this form usually coincides with exacerbation of systemic manifestations developing a prolonged disease activity and may resemble a drug eruption or can simulate toxic epidermal necrolysis. The incidence of this generalized form is estimated to be approximately in 5-10% of patients with systemic lupus erythematosus (SLE) (Cardinali et al., 2000; Fabbri et al., 2003;

Lesions Face Aids
Figure 1. Acute cutaneous lupus erythematosus (ACLE). Confluent, erythematous, hyperkeratotic lesions on the face of a patient with a generalized form.

Sontheimer, 1997). It is characterized by a symmetrically distributed maculopapular or ex-anthematous eruption with a pruritic component. The colour of the lesions is usually red or, less frequently, dull red or livid, and there have been reports of patients presenting with severe involvement of the oral mucosa or the palms and phalanges (Braverman, 1981; McCauliffe, 2001).

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