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Acute Cutaneous Lupus Erythematosus (ACLE) Clinical Presentation

  • Author: Ivan D Camacho, MD; Chief Editor: William D James, MD  more...
Updated: Nov 12, 2015


Primary lesions in acute cutaneous lupus erythematosus include the following[3] :

  • 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.[4]


Physical Examination

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.

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, 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.[5]

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.[6] (See the image below.)

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

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.[7]

Contributor Information and Disclosures

Ivan D Camacho, MD Dermatologist, Private Practice; Voluntary Assistant Professor of Dermatology, Department of Dermatology and Cutaneous Surgery, University of Miami, Leonard M Miller School of Medicine

Ivan D Camacho, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Florida Medical Association, International Society of Dermatology, Women's Dermatologic Society

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.


Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and American College of Rheumatology

Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Electrical Optical Sciences Consulting fee Consulting; Celgene Honoraria Safety Monitoring Committee; GSK - Glaxo Smith Kline Consulting fee Consulting; TenXBioPharma Consulting fee Safety Monitoring Committee

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

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Relationship of acute cutaneous lupus erythematosus (ACLE) to systemic disease. LE is lupus erythematosus. CCLE is chronic cutaneous lupus erythematosus. SCLE is subacute cutaneous lupus erythematosus.
Erythema involving the malar area, forehead, and neck. Note sparing of some of the creases.
Toxic epidermal necrolysis–like eruption.
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