Acute Cutaneous Lupus Erythematosus (ACLE) Clinical Presentation

Updated: Jul 20, 2021
  • Author: Fnu Nutan, MD, FACP; Chief Editor: William D James, MD  more...
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Presentation

History

Acute cutaneous lupus erythematosus (ACLE) can be classified into the following three categories:

  • Localized ACLE
  • Generalized ACLE
  • Toxic epidermal necrolysis (TEN)–like ACLE

Primary lesions in ACLE therefore include the classic facial malar rash, confluent erythema and edema, erythematous macules and papules that eventually become confluent, morbilliform macules and papules in a generalized photo-distributed pattern, bullous lesions resembling TEN, and erythema multiforme–like lesions (Rowell syndrome). [13]

The facial rash of ACLE includes the malar area and the cheeks and always spares the nasolabial folds. Other sites of involvement include the forehead, periorbital area, and sides of the neck, essentially all the areas that are exposed to the sun.

Generalized ACLE, although less common, presents as a erythematous morbilliform rash on exposed parts of the body, such as the extensor surfaces of the hand. Classically, it spares the knuckles.

Sometimes, vesicles and bullae on erythematous skin resembling Stevens-Johnson syndrome (SJS)/TEN (SJS/TEN) can be seen because the inflammatory infiltrate is so severe. [14]

Superficial oral ulcers of the posterior surface of the hard palate are seen most commonly. Occasionally, buccal and gingival mucosae and the tongue may be involved.

Note that ACLE may coexist with other lupus erythematosus–specific skin diseases. In about 20% of cases, ACLE and subacute cutaneous lupus erythematosus (SCLE) coexist. However, the occurrence of ACLE with chronic cutaneous lupus erythematosus (CCLE) is unusual.

Erythema multiforme–like lesions may be seen with ACLE and SCLE and is often referred to as Rowell syndrome when associated with immunologic serum abnormalities such as a speckled antinuclear antibody (ANA) pattern and positive rheumatoid factor. [15]

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Physical Examination

The most common presentation of ACLE is a red macular eruption involving the malar area (see image below). The forehead, periorbital area, and neck also may be involved, representing a photodistribution. Occasionally, unilateral involvement may occur.

Erythema involving the malar area, forehead, and n Erythema involving the malar area, forehead, and neck. Note sparing of some of the creases.

Less commonly, ACLE presents as a generalized photosensitive eruption, while more rarely, patients present with widespread blistering simulating SJS)/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. [16]

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. [17] See the image below.

Toxic epidermal necrolysis–like eruption. Toxic epidermal necrolysis–like eruption.

Patients with ACLE 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. [18]

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Complications

Unlike discoid lupus lesions, lesions of ACLE do not scar with healing. Transient hyperpigmentation is seen during the healing phase. Oral lesions heal without scarring. Very rarely, hypopigmentation can be seen post healing of the malar rash.

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