Acute Cutaneous Lupus Erythematosus (ACLE) Clinical Presentation
- Author: Ivan D Camacho, MD; Chief Editor: William D James, MD more...
Primary lesions in acute cutaneous lupus erythematosus include the following :
Confluent erythema and edema
Erythematous macules and papules that eventually become confluent
Bullous lesions resembling TEN
Morbilliform macules and papules in a generalized, photo-distributed pattern
Erythema multiforme–like lesions
The malar eminence (representing the wings of the butterfly) and the nasal bridge (representing the body of the butterfly) typically are involved. Other sites of involvement include the forehead, periorbital area, and sides of the neck. Occasionally, a generalized photo-induced eruption may occur.
Associated findings include superficial ulceration primarily involving the posterior surface of the hard palate. Occasionally, buccal and gingival mucosae and the tongue may be involved.
Note that acute cutaneous lupus erythematosus may coexist with other lupus erythematosus–specific skin diseases. Localized acute cutaneous lupus erythematosus lesions have been observed in 20% of subacute cutaneous lupus erythematosus patients; however, the occurrence of acute cutaneous lupus erythematosus with chronic cutaneous lupus erythematosus is unusual.
Erythema multiforme–like lesions may be seen with acute and subacute cutaneous lupus erythematosus, often referred to as Rowell syndrome when associated with immunologic serum abnormalities such as a speckled antinuclear antibody (ANA) pattern and positive rheumatoid factor.
The most common presentation of acute cutaneous lupus erythematosus is a red macular eruption involving the malar area. (See the image below.) The forehead, periorbital area, and neck also may be involved, representing a photodistribution. Occasionally, unilateral involvement may occur.
Less commonly, acute cutaneous lupus erythematosus presents as a generalized photosensitive eruption, while more rarely, patients present with widespread blistering simulating Stevens-Johnson Syndrome (SJS) /Toxic epidermal necrolysis (TEN). SJS/TEN-like cutaneous lupus erythematosus is due to extensive epidermal necrosis (which is believed to be a phototoxic reaction and may be triggered by intensive ultraviolet exposure) and must be differentiated from drug-induced TEN occurring in a patient with lupus erythematosus. The combination of recent lupus exacerbation, photodistribution, annular lesions, absent or mild focal erosive mucosal involvement and histological changes including junctional vacuolar alteration, solitary necrotic keratinocytes at lower epidermal levels, dense periadnexal and perivascular lymphocytic infiltrates, and mucin favor lupus erythematosus over SJS or TEN.
The term acute syndrome of apoptotic pan-epidermolysis (ASAP) has been proposed for the TEN-like cutaneous injury pattern that can occur in settings of lupus erythematosus, where Fas-Fas ligand interactions are implicated in the massive keratinocyte apoptosis. (See the image below.)
Patients with acute cutaneous lupus erythematosus frequently experience superficial ulceration of the oral and nasal mucosae. These lesions may produce extreme discomfort in some patients, although the lesions may be entirely painless in others. The posterior surface of the hard palate is the site affected most frequently; however, the gingival, buccal, and lingual mucosae also may be involved.
An unusual reported cutaneous presentation is the presence of erythematous, slightly scaly, pruritic papules and plaques on the elbows.
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