Acute Cutaneous Lupus Erythematosus

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The typical clinical manifestations of ACLE are characterized by a localized erythema known as the "malar rash" or "butterfly rash" on the central portion of the face or by a generalized, more widespread form (Fabbri et al. 2003, Sontheimer and Provost 2003). Localized ACLE may only affect the skin transiently, and the lesions may last for only several days up to a few weeks. Therefore, at the onset of disease, the patients may mistake this rash for sunburn and may seek medical advice only after the lesions have persisted for a longer period. Generalized ACLE, also known as "photosensitve lupus rash", is a less common variety and may be located anywhere on the body; however, it has a predilection for sun-exposed areas of the face, extensor aspects of the arms and forearms, and the dorsal aspects of the hands. It generally presents as a maculopapular or exanthematous eruption with a pruritic component. In most of the patients, systemic manifestation is strongly associated with ACLE, preceding by weeks or months the onset of a multisystem disease along with the confirmatory serologic findings (Watanabe and Tsuchida 1995, Wysenbeek et al. 1992, Yung and Oakley 2000). Since dermatologists are not usually the primary managers of such patients, few data concerning this form are available in the dermatologic literature.

ACLE has been reported in 20%-60% of large lupus patient cohorts, and it is more common in women than in men (Cervera et al. 1993,Pistiner et al. 1991,Wysenbeek et al. 1992).In one study,women were found to be six times more often affected than men, and the patients were on average in their second or third decade of life (Ng et al. 2000). Ultraviolet (UV) exposure is a common exogenous factor to be capable of precipitating ACLE (Kuhn et al. 2001a,Wysenbeek et al. 1989),and photosensitive patients sometimes report an exacerbation of their systemic symptoms after sun exposure. Furthermore, infections, especially with subtle types of viruses, or certain drugs, e. g., hydralazine, isoniazide, and procainamide, have also been found to induce or aggravate this disease (Pramatarov 1998,Rubin 1999).A possible association withHLA-DR2 and -DR3 has been suspected, and familial associations or concordance in twins suggest a genetic component.

Fig. 6.1. Localized acute cutaneous lupus erythematous (ACLE). Classic butterfly rash characterized by symmetrical erythema on the malar areas of the face

Fig. 6.1. Localized acute cutaneous lupus erythematous (ACLE). Classic butterfly rash characterized by symmetrical erythema on the malar areas of the face

Transient Maculopapular Butterfly Rash

Fig. 6.2. Localized acute cutaneous lupus erythematous (ACLE). Erythematous lesions on the face of a patient that became confluent and hyper-

The localized form of ACLE usually begins with small, discrete erythematous macules and papules, occasionally associated with fine scales involving both the malar areas and the bridge of the nose while sparing the nasolabial folds (Fig. 6.1). This classic "malar rash" or" butterfly rash" can disappear without scarring and pigmentation or gradually becomes confluent and hyperkeratotic (Fig. 6.2), and facial swelling may be severe in some patients with this disease (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. Furthermore, patients with ACLE may have diffuse thinning or a receding frontal hairline with broken hairs (lupus hair), telangiectasias and erythema of the proximal nail fold, and cuticular abnormalities (Patel and Werth 2002). Superficial ulcerations of the oral and/or nasal mucosa are frequently accompanied with ACLE and may cause extreme discomfort in some patients. The posterior areas of the hard palate are most commonly affected; however, the gingival, buccal, and lingual mucosa may also be involved. In general, ACLE lesions are nonscarring, and the simultaneous occurrence of ACLE and other variants of CLE, such as DLE, is uncommon.

Some patients experience an extremely acute, generalized form of ACLE that presents as a maculopapular rash and can develop a more prolonged disease activity (Fabbri et al. 2003, Sontheimer 1997). In the few existing reports in the literature, this form is characterized by a generalized eruption of symmetrically distributed small,

Fig. 6.2. Localized acute cutaneous lupus erythematous (ACLE). Erythematous lesions on the face of a patient that became confluent and hyper-


Subacute Cutaneous Lupus Erythematosus
Fig. 6.3. Generalized acute cutaneous lupus erythematous (ACLE). Erythematous plaques over the dorsal aspects of the hands in a patient with severe SLE
Facial Edema Urticaria Lupus

confluent erythematous macules and papules with a pruritic component. The color 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 (Fig. 6.3) (Braverman 1981,McCauliffe 2001). In contrast to the classic malar erythema of ACLE, the generalized form is a rather uncommon cutaneous manifestation that may be located anywhere on the body, although the preferred sites are above the waistline (Sontheimer and Provost 2003, Yell et al. 1996). It may resemble a drug eruption or can simulate toxic epidermal necrolysis, and it frequently occurs after sun exposure. As with "malar rash" or "butterfly rash", the onset of the generalized form usually coincides with exacerbation of systemic disease. Its incidence is estimated to be approximately 5%-10% of patients with SLE (Cardinali et al. 2000, Tan et al. 1982), but the sporadic observations published in the dermato-logic literature probably underestimate the real prevalence of this from.

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