Acute Cutaneous Lupus Erythematosus (ACLE) Clinical Presentation

  • Author: Ivan D Camacho, MD; Chief Editor: William D James, MD   more...
 
Updated: Nov 17, 2011
 

History

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

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.

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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 nErythema 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 TEN. TEN-like cutaneous lupus erythematosus (which is believed to be a phototoxic reaction and may be triggered by intensive ultraviolet exposure) must be differentiated from drug-induced TEN occurring in a patient with lupus erythematosus. 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.[3] (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.

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Contributor Information and Disclosures
Author

Ivan D Camacho, MD  Assistant Professor of Dermatology, Department of Dermatology and Cutaneous Surgery, University of Miami, Leonard M Miller School of Medicine; Medical Director of Dermatology Clinic, Jackson Memorial Hospital

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, and Women's Dermatologic Society

Disclosure: Nothing to disclose.

Coauthor(s)

Charmaine Browne, MD, FRCP(C)  Clinical Assistant Professor, Department of Dermatology, University of Texas Southwestern Medical Center at Dallas; Clinical Assistant Professor, Department of Dermatology, University of Texas Health Science Center at San Antonio

Charmaine Browne, MD, FRCP(C) is a member of the following medical societies: American Academy of Dermatology, American Contact Dermatitis Society, International Society of Dermatology, Royal College of Physicians and Surgeons of Canada, Society for Pediatric Dermatology, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD  Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System

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

Disclosure: Elsevier Royalty Other

Additional Contributors

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.

References

  1. Renner R, Sticherling M. The different faces of cutaneous lupus erythematosus. G Ital Dermatol Venereol. Apr 2009;144(2):135-47. [Medline].

  2. Moghadam-Kia S, Chilek K, Gaines E, et al. Cross-sectional analysis of a collaborative Web-based database for lupus erythematosus-associated skin lesions: prospective enrollment of 114 patients. Arch Dermatol. Mar 2009;145(3):255-60. [Medline].

  3. Ting W, Stone MS, Racila D, Scofield RH, Sontheimer RD. Toxic epidermal necrolysis-like acute cutaneous lupus erythematosus and the spectrum of the acute syndrome of apoptotic pan-epidermolysis (ASAP): a case report, concept review and proposal for new classification of lupus erythematosus vesiculobullous skin lesions. Lupus. 2004;13(12):941-50. [Medline].

  4. Jerdan MS, Hood AF, Moore GW, Callen JP. Histopathologic comparison of the subsets of lupus erythematosus. Arch Dermatol. Jan 1990;126(1):52-5. [Medline].

  5. Cortés-Hernández J, Torres-Salido M, Castro-Marrero J, Vilardell-Tarres M, Ordi-Ros J. Thalidomide in the treatment of refractory cutaneous lupus: prognostic factors of clinical outcome. Br J Dermatol. Oct 16 2011;[Medline].

  6. Goodfield M, Davison K, Bowden K. Intravenous immunoglobulin (IVIg) for therapy-resistant cutaneous lupus erythematosus (LE). J Dermatolog Treat. Jan 2004;15(1):46-50. [Medline].

  7. Kok MR, Vos K, Bos JD, Tak PP. Remission of incapacitating acute cutaneous lupus erythematosus in a patient with systemic lupus erythematosus by B cell-depletive therapy. J Clin Rheumatol. Oct 2010;16(7):345. [Medline].

  8. Uthman I, Taher A, Abbas O, Menassa J, Ghosn S. Successful treatment of refractory skin manifestations of systemic lupus erythematosus with rituximab: report of a case. Dermatology. 2008;216(3):257-9. [Medline].

  9. Pisoni CN, Obermoser G, Cuadrado MJ, et al. Skin manifestations of systemic lupus erythematosus refractory to multiple treatment modalities: poor results with mycophenolate mofetil. Clin Exp Rheumatol. May-Jun 2005;23(3):393-6. [Medline].

  10. Simsek I, Cinar M, Erdem H, Pay S, Meric C, Dinc A. Efficacy of plasmapheresis in the treatment of refractory toxic epidermal necrolysis-like acute cutaneous lupus erythematosus. Lupus. 2008;17(6):605-6. [Medline].

  11. Kuhn A, Gensch K, Haust M, et al. Efficacy of tacrolimus 0.1% ointment in cutaneous lupus erythematosus: a multicenter, randomized, double-blind, vehicle-controlled trial. J Am Acad Dermatol. Jul 2011;65(1):54-64, 64.e1-2. [Medline].

  12. Díez MT, Boixeda P, Moreno C, González JA, Zamorano ML, Olasolo PJ. Histopathology and immunohistochemistry of cutaneous lupus erythematosus after pulsed dye laser treatment. Dermatol Surg. Jul 2011;37(7):971-81. [Medline].

  13. Kuhn A, Ruland V, Bonsmann G. Photosensitivity, phototesting, and photoprotection in cutaneous lupus erythematosus. Lupus. Aug 2010;19(9):1036-46. [Medline].

  14. Ding C, Foote S, Jones G. B-cell-targeted therapy for systemic lupus erythematosus: an update. BioDrugs. 2008;22(4):239-49. [Medline].

  15. ZZZ.

 
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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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