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Acute Cutaneous Lupus Erythematosus
(ACLE)
Updated: Nov 12, 2015
Author: Ivan D Camacho, MD; Chief Editor: William D James, MD more...
OVERVIEW
Background
Lupus erythematosus is a heterogeneous connectivetissue disease associated with polyclonal Bcell
activation and is believed to result from the interplay of genetic, environmental, and hormonal factors.
The spectrum of disease involvement can vary from limited cutaneous involvement to devastating
systemic disease. (See Etiology.)
From a dermatologic standpoint, the type of skin involvement can prove to be a good barometer of the
pattern of underlying systemic activity. Lupus erythematosus–specific skin diseases are recognized in
3 categories, including (1) acute cutaneous lupus erythematosus (ACLE), (2) subacute cutaneous
lupus erythematosus (SCLE), and (3) chronic cutaneous lupus erythematosus (CCLE). (See the
diagram below.) Clinical characteristics of each group are unique, although histopathologically, only
subtle differences are identified. The focus of this article is on acute cutaneous lupus erythematosus.
[1, 2] (See Etiology, History, Physical Examination, and Workup.)
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.
http://emedicine.medscape.com/article/1065292overview 1/7
3/29/2017 Acute Cutaneous Lupus Erythematosus (ACLE): Background, Etiology, Epidemiology
View Media Gallery
See Cutaneous Clues to Accurately Diagnosing Rheumatologic Disease, a Critical Images slideshow,
to help recognize cutaneous manifestations of rheumatologic diseases.
Acute cutaneous lupus erythematosus refers to a typical malar eruption in a butterfly pattern localized
to the central portion of the face and/or a more generalized maculopapular eruption representing a
photosensitive dermatitis. (See the image below.) The condition has a strong association with the
systemic disease for which patients present to rheumatologists and internists. (See History and
Physical Examination.)
Erythema involving the malar area, forehead, and neck. Note sparing of some of the creases.
View Media Gallery
Acute cutaneous lupus erythematosus can be transient, lasting for several days to weeks. Lesions
wax and wane with sun exposure over a period of several hours; however, some patients experience
prolonged disease activity.
Resolution of lesions may result in postinflammatory hyperpigmentation, especially in patients with
darkly pigmented skin. Usually, the lesions are nonscarring. (See Prognosis, History, and Physical
Examination.)
Patient education
Educate patients about the nature of skin, which acts as a barometer of disease activity. Control of the
cutaneous manifestations depends ultimately on overall control of the disease. Instruct patients
regarding the effects of ultraviolet light in exacerbating the disease.
For patient education information, see the Arthritis Center, as well as Lupus (Systemic Lupus
Erythematosus).
Etiology
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The etiology of lupus erythematosus is believed to be multifactorial, involving genetic, environmental,
and hormonal factors. An association with human leukocyte antigen DR2 and human leukocyte
antigen DR3 has been identified. Concordance in monozygotic twins and familial associations support
a genetic basis in acute cutaneous lupus erythematosus.
More than 25 genes have been identified as contributing to the mechanisms that predispose patients
to lupus. They include alleles in the major histocompatibility complex region (multiple genes): IRF5,
ITGAM, STAT4, BLK, BANK1, PDCD1, PTPN22, TNFSF4, TNFA1P3, SPP1, some fc gene receptors,
and deficiency in several complement components, including C1qC4+C2.
In patients who are predisposed genetically, exposure to natural ultraviolet radiation is a frequent
precipitating factor for lupus erythematosus. In addition, certain viruses (eg, EpsteinBarr virus,
cytomegalovirus, human immunodeficiency virus [HIV]) have been implicated in precipitating or
exacerbating lupus erythematosus in genetically predisposed individuals.
Chemicals such as Lcanavanine, which is present in alfalfa sprouts, have been known to induce
systemic lupus erythematosus–like illness. Drugs implicated in inducing a lupus erythematosus–like
illness (eg, procainamide, isoniazid, hydralazine) typically do not induce acute cutaneous lupus
erythematosus.
See also Bullous Lupus Erythematosus, Discoid Lupus Erythematosus, DrugInduced Lupus
Erythematosus, and Subacute Cutaneous Lupus Erythematosus.
Immunopathology
Data concerning direct immunofluorescence in acute cutaneous lupus erythematosus are sparse. In
one study, the results of 5 (100%) of 5 skin biopsy specimens were reported as positive for the lupus
band test. The lupus band test reveals the presence of immunoglobulins and C3 complement
components along the dermoepidermal junction. All 3 immunoglobulin classes (immunoglobulin G
[IgG], IgM, IgA) and a variety of complement components have been identified at the dermoepidermal
junction.
Research has shown that 60% of patients with a malar eruption of lupus erythematosus have positive
lupus band test results. In nonlesional skin, positive lupus band test results correlate strongly with an
aggressive course of systemic disease.
Epidemiology
In the United States, the malar rash has been reported in 2060% of patients in large lupus
erythematosus cohorts, while limited data suggest that the maculopapular eruption is present in 35%
of patients with systemic lupus erythematosus. The malar rash is believed to be associated with a
younger age of disease onset.
Racerelated demographics
Precise data concerning the prevalence of acute cutaneous lupus erythematosus in specific racial
groups are not available; however, since photosensitivity is observed more frequently in whites than in
blacks, the same prevalence of acute cutaneous lupus erythematosus may be inferred. Estimates
suggest that 1 in 250 black women in the United States and the Caribbean and 1 in 1000 Chinese
persons have systemic lupus erythematosus. Although lupus erythematosus may be rare in most
parts of Africa, data concerning this finding conflict.
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Data concerning acute cutaneous lupus erythematosus are difficult to interpret, since a lack of
conformity is found in the description of lesions, and biopsy data are lacking for skin lesions observed
in patients with systemic disease.
Prognosis
Significant morbidity and potential mortality are associated with systemic lupus erythematosus, of
which acute cutaneous lupus erythematosus is a manifestation.
The malar eruption tends to wax and wane with systemic activity; however, whether the presence of
malar rash indicates a worse overall outlook for patients has not been determined. Progression to
lupus erythematosus–specific bullous dermatosis (aggravated by sun exposure) may occur, producing
a toxic epidermal necrolysis (TEN) ̶ type picture.
No definite correlation has been identified between acute cutaneous lupus erythematosus and
nephritis; however, localized lesions of acute cutaneous lupus erythematosus are believed to tend to
wax and wane, paralleling the underlying systemic disease. Postinflammatory hyperpigmentation may
occur in darkskinned patients following resolution.
Clinical Presentation
References
1. Renner R, Sticherling M. The different faces of cutaneous lupus erythematosus. G Ital Dermatol
Venereol. 2009 Apr. 144(2):13547. [Medline].
2. MoghadamKia S, Chilek K, Gaines E, et al. Crosssectional analysis of a collaborative Web
based database for lupus erythematosusassociated skin lesions: prospective enrollment of 114
patients. Arch Dermatol. 2009 Mar. 145(3):25560. [Medline]. [Full Text].
3. Parodi A, Cozzani E. Cutaneous manifestations of lupus erythematosus. G Ital Dermatol
Venereol. 2014 Oct. 149 (5):54954. [Medline].
4. Torchia D, Romanelli P, Kerdel FA. Erythema multiforme and StevensJohnson syndrome/toxic
epidermal necrolysis associated with lupus erythematosus. J Am Acad Dermatol. 2012.
67(3):41721. [Medline].
5. Ziemer M, Kardaun SH, Liss Y, Mockenhaupt M. StevensJohnson syndrome and toxic
epidermal necrolysis in patients with lupus erythematosus: a descriptive study of 17 cases from
a national registry and review of the literature. Br J Dermatol. 2012. 166(3):575600. [Medline].
6. Ting W, Stone MS, Racila D, Scofield RH, Sontheimer RD. Toxic epidermal necrolysislike 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):94150. [Medline].
7. Bielsa I, Guinovart RM, FernándezFigueras MT, Rodríguez C, Ferrándiz C. Cutaneous lupus
erythematosus on the elbows: a peculiar localization. 2012. 21(1):848. [Medline].
8. Jerdan MS, Hood AF, Moore GW, Callen JP. Histopathologic comparison of the subsets of lupus
erythematosus. Arch Dermatol. 1990 Jan. 126(1):525. [Medline].
9. Böckle BC, Stanarevic G, Sepp NT. Detection of Ro/SSA antibodies in lupus erythematosus:
what does it mean for the dermatologist?. J Am Acad Dermatol. 2013 Mar. 68(3):38594.
http://emedicine.medscape.com/article/1065292overview 4/7
3/29/2017 Acute Cutaneous Lupus Erythematosus (ACLE): Background, Etiology, Epidemiology
[Medline].
10. CortésHernández J, TorresSalido M, CastroMarrero J, VilardellTarres M, OrdiRos J.
Thalidomide in the treatment of refractory cutaneous lupus: prognostic factors of clinical
outcome. Br J Dermatol. 2011 Oct 16. [Medline].
11. Raptopoulou A, Linardakis C, Sidiropoulos P, Kritikos HD, Boumpas DT. Pulse
cyclophosphamide treatment for severe refractory cutaneous lupus erythematosus. Lupus.
2010. 19(6):7447. [Medline].
12. Goodfield M, Davison K, Bowden K. Intravenous immunoglobulin (IVIg) for therapyresistant
cutaneous lupus erythematosus (LE). J Dermatolog Treat. 2004 Jan. 15(1):4650. [Medline].
13. Kok MR, Vos K, Bos JD, Tak PP. Remission of incapacitating acute cutaneous lupus
erythematosus in a patient with systemic lupus erythematosus by B celldepletive therapy. J Clin
Rheumatol. 2010 Oct. 16(7):345. [Medline].
14. 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):2579. [Medline].
15. Vital EM, Wittmann M, Edward S, Md Yusof MY, MacIver H, Pease CT, et al. Brief report:
responses to rituximab suggest B cellindependent inflammation in cutaneous systemic lupus
erythematosus. Arthritis Rheumatol. 2015 Jun. 67 (6):158691. [Medline].
16. Ky C, Swasdibutra B, Khademi S, Desai S, Laquer V, Grando SA. Efficacy of Intravenous
Immunoglobulin Monotherapy in Patients with Cutaneous Lupus Erythematosus: Results of
ProofofConcept Study. Dermatol Reports. 2015 Mar 16. 7 (1):5804. [Medline].
17. 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. 2005 MayJun. 23(3):3936. [Medline].
18. Simsek I, Cinar M, Erdem H, Pay S, Meric C, Dinc A. Efficacy of plasmapheresis in the
treatment of refractory toxic epidermal necrolysislike acute cutaneous lupus erythematosus.
Lupus. 2008. 17(6):6056. [Medline].
19. Kuhn A, Gensch K, Haust M, et al. Efficacy of tacrolimus 0.1% ointment in cutaneous lupus
erythematosus: a multicenter, randomized, doubleblind, vehiclecontrolled trial. J Am Acad
Dermatol. 2011 Jul. 65(1):5464, 64.e12. [Medline].
20. 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. 2011 Jul. 37(7):97181. [Medline].
21. Kuhn A, Ruland V, Bonsmann G. Photosensitivity, phototesting, and photoprotection in
cutaneous lupus erythematosus. Lupus. 2010 Aug. 19(9):103646. [Medline].
22. Ding C, Foote S, Jones G. Bcelltargeted therapy for systemic lupus erythematosus: an update.
BioDrugs. 2008. 22(4):23949. [Medline].
Media Gallery
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.
http://emedicine.medscape.com/article/1065292overview 5/7
3/29/2017 Acute Cutaneous Lupus Erythematosus (ACLE): Background, Etiology, Epidemiology
Erythema involving the malar area, forehead, and neck. Note sparing of some of the creases.
Toxic epidermal necrolysis–like eruption.
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Contributor Information and Disclosures
Author
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, ViceChairman, 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: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for:
nakedbiome<br/>Received income in an amount equal to or greater than $250 from:
elsevier;webMD<br/>editor in chief for: statpearls.
Acknowledgements
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
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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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