Acute Cutaneous Lupus Erythematosus (ACLE) Workup

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

Approach Considerations

Because acute cutaneous lupus erythematosus and systemic lupus erythematosus are associated closely, it is safe to assume that the laboratory findings in systemic lupus erythematosus closely mirror the findings in acute cutaneous lupus erythematosus.

Diagnostic data from laboratory tests are supported by histopathologic examination of the skin. Further diagnostic substantiation is obtained by performing immunofluorescent examination of skin lesions.

The most striking histologic change in acute cutaneous lupus erythematosus is the presence of edema involving upper dermis and focal liquefactive degeneration of the basal cell layer. Cellular dermal infiltrate is sparse and consists of lymphocytes. In extreme cases, dissolution of the basal layer occurs secondary to extensive vacuolization, forming a subepidermal bulla.[4]

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Laboratory Studies

As previously mentioned, ANA assay results invariably are positive in patients with systemic lupus erythematosus and, therefore, in patients with acute cutaneous lupus erythematosus. The peripheral rim pattern is associated most strongly with lupus erythematosus, although other patterns commonly are present. ANA results are less likely to be positive in dermatomyositis, which mimics lupus erythematosus both clinically and histologically.

Anti–double-stranded deoxyribonucleic acid (DNA) antibody (anti-dsDNA) assay is specific for systemic lupus erythematosus and is present in 60-80% of patients with acute cutaneous lupus erythematosus, often in high titers.

Complement levels usually are depressed in patients with acute cutaneous lupus erythematosus.

Anti-Sm antibody assay has a strong specificity for systemic lupus erythematosus; therefore, perform this assay to exclude underlying systemic involvement. This is particularly relevant in patients in whom anti-dsDNA results are negative.

Low-specificity tests include the following:

  • U1 ribonucleoprotein antibody assay - Results are positive in mixed connective-tissue disease, which sometimes manifests as a malar eruption
  • Complete blood count (CBC) - Anemia, leukopenia, and/or thrombocytopenia may be seen in patients with acute cutaneous lupus erythematosus who have systemic involvement
  • Erythrocyte sedimentation rate - Although a nonspecific marker, marked elevations in levels indicate possible systemic involvement
  • Urinalysis - Proteinuria, hematuria, and urine casts are indicative of underlying nephritis
  • Creatinine and blood urea nitrogen (BUN) levels - Elevation indicates renal compromise
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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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