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Cutaneous lupus erythematosus: Diagnosis and treatment

https://doi.org/10.1016/j.berh.2013.07.008Get rights and content

Abstract

Cutaneous lupus erythematosus (CLE) encompasses a wide range of dermatologic manifestations, which may or may not be associated with the development of systemic disease. Cutaneous lupus is divided into several sub-types, including acute CLE (ACLE), sub-acute CLE (SCLE) and chronic CLE (CCLE). CCLE includes discoid lupus erythematosus (DLE), LE profundus (LEP), chilblain cutaneous lupus and lupus tumidus. The diagnosis of these diseases requires proper classification of the sub-type, through a combination of physical examination, laboratory studies, histology, antibody serology and occasionally direct immunofluorescence, while ensuring to exclude systemic disease. The treatment of cutaneous lupus consists of patient education on proper sun protection along with appropriate topical and systemic agents. Systemic agents are indicated in cases of widespread, scarring or treatment-refractory disease. In this chapter, we discuss issues in classification and diagnosis of the various sub-types of CLE, as well as provide an update on therapeutic management.

Introduction

The auto-immune disease lupus erythematosus (LE) is associated with a broad range of cutaneous pathology. Cutaneous manifestations are frequently the presenting sign of LE, and in the case of certain cutaneous LE (CLE) sub-types, they can occur in the absence of systemic disease. CLE is two to three times more frequent than systemic lupus erythematosus (SLE) [1]. Similar to proposed aetiologies for SLE, current theories discuss a multifactorial relationship leading to the development of cutaneous lupus, including genetic susceptibility, auto-immune induction and immune-system damage.

Section snippets

How is CLE currently classified?

At present, dermatologists use the only universally accepted criteria for the classification of SLE, which was set forth by the American College of Rheumatology (ACR). This scheme of 11 clinical and lab criteria was developed by rheumatologists for the purpose of distinguishing SLE from other auto-immune diseases. The ACR guidelines require four of 11 criteria to be met for a diagnosis of SLE; however, four of the criteria are cutaneous in nature (malar rash, discoid lesions, mucosal ulcers and

Acute cutaneous lupus erythematosus

ACLE typically presents in the third decade of life and is frequently associated with active SLE [9], [10]. There are localised and generalised forms of ACLE. The localised form is the frequently described malar, or ‘butterfly’ rash, which refers to erythema that occurs over both cheeks, extends over the nasal bridge and spares the nasolabial folds [11]. These lesions are classically transient, sun-induced and non-scarring, although dyspigmentation can occur [12]. Patients may initially mistake

How can we properly diagnose CLE?

In order to properly diagnose cutaneous manifestations of LE, the physician must first correctly classify the sub-type and exclude systemic involvement of the disease. As discussed earlier, diagnosis based solely on ACR criteria should be avoided, as the ACR criteria were designed to distinguish between the various auto-immune diseases. Rather, CLE diagnosis should be based on the findings of patient history, clinical examination, laboratory studies, serology as well as histology and DIF

Prevention

In treating CLE, dermatologists aim at preventing the formation and progression of lesions and improving skin appearance through a combination of patient education and topical and systemic therapies (Fig. 4). Patient education on heat, sun and drug avoidance is standard. Patients should be advised to avoid manipulation of lesions, as this can induce new lesions [12]. Makeup products such as Dermablend or Covermark should be offered to camouflage lesions. Strict sunscreen adherence is a critical

Summary

CLE comprises a range of dermatologic manifestations, including ACLE, SCLE and CCLE. The ACR criteria, which include four cutaneous signs, may lead to overdiagnosis of SLE in patients with predominantly cutaneous disease. The diagnosis of CLE requires proper classification of the sub-type, which is best accomplished by a focus on the clinical and histologic findings. Serology and DIF are less helpful in making the diagnosis. CLE treatment combines sun protection, topical therapies and systemic

References (83)

  • D. Thaci et al.

    Malignancy concerns of topical calcineurin inhibitors for atopic dermatitis: facts and controversies

    Clinics in Dermatology

    (2010)
  • A. Erceg et al.

    Efficacy and safety of pulsed dye laser treatment for cutaneous discoid lupus erythematosus

    Journal of American Academy of Dermatology

    (2009)
  • E.W. Piette et al.

    Impact of smoking in cutaneous lupus erythematosus

    Archives of Dermatology

    (2012)
  • M.F. Marmor et al.

    Recommendations on screening for chloroquine and hydroxychloroquine retinopathy: a report by the American Academy of Ophthalmology

    Ophthalmology

    (2002)
  • A. Kuhn et al.

    Cutaneous lupus erythematosus: update of therapeutic options: part 2

    Journal of American Academy of Dermatology

    (2011)
  • R. Ashinoff et al.

    Resistant discoid lupus erythematosus of palms and soles: successful treatment with azathioprine

    Journal of American Academy of Dermatology

    (1988)
  • S. Bastuji-Garin et al.

    Incidence and risk factors for thalidomide neuropathy: a prospective study of 135 dermatologic patients

    Journal of Investigative Dermatology

    (2002)
  • I. Braunstein et al.

    Lenalidomide therapy in treatment-refractory cutaneous lupus erythematosus: histologic and circulating leukocyte profile and potential risk of a systemic lupus flare

    Journal of American Academy of Dermatology

    (2012)
  • A. Kuhn et al.

    Alitretinoin for cutaneous lupus erythematosus

    Journal of American Academy of Dermatology

    (2012 Sep)
  • B. Tebbe et al.

    Epidemiology and socioeconomic impact of skin disease in lupus erythematosus

    Lupus

    (1997)
  • J. Albrecht et al.

    Dermatology position paper on the revision of the 1982 ACR criteria for systemic lupus erythematosus

    Lupus

    (2004)
  • C.M. Gronhagen et al.

    Cutaneous lupus erythematosus and the association with systemic lupus erythematosus: a population-based cohort of 1088 patients in Sweden

    British Journal of Dermatology

    (2011)
  • I.T. Wieczorek et al.

    Progression from cutaneous to systemic lupus erythematosus by ACR criteria usually occurs with mild disease and few systemic symptoms

    Journal of Investigative Dermatology (Abstract)

    (2012)
  • M. Petri et al.

    Derivation and validation of systemic lupus international collaborating clinics classification criteria for systemic lupus erythematosus

    Arthritis and Rheumatism

    (2012)
  • A. Kuhn et al.

    Classification of cutaneous lupus erythematosus

  • P.P. Ng et al.

    Epidemiology of cutaneous lupus erythematosus in a tertiary referral centre in Singapore

    Australian Journal of Dermatology

    (2000)
  • P. Fabbri et al.

    Cutaneous lupus erythematosus: diagnosis and management

    American Journal of Clinical Dermatology

    (2003)
  • H.W. Walling et al.

    Cutaneous lupus erythematosus: issues in diagnosis and treatment

    American Journal of Clinical Dermatology

    (2009)
  • L.A. Lee et al.

    Lupus erythematosus

  • P. Patel et al.

    Cutaneous lupus erythematosus: a review

    Clinics in Dermatology

    (2002)
  • N.R. Rowell et al.

    Lupus erythematosus and erythema multiforme-like lesions

    Archives of Dermatology

    (1963)
  • B. Tebbe et al.

    Markers in cutaneous lupus erythematosus indicating systemic involvement. A multicenter study on 296 patients

    Acta Dermato Venereologica

    (1997)
  • J.P. Callen et al.

    Subacute cutaneous lupus erythematosus: clinical, serologic, immunogenetic, and therapeutic considerations in seventy-two patients

    Arthritis and Rheumatism

    (1988)
  • A. Parodi et al.

    Clinical, histological and immunopathological features of 58 patients with subacute cutaneous lupus erythematosus: a review by the Italian group of immunodermatology

    Dermatology

    (2000)
  • S. Moghadam-Kia et al.

    Cross-sectional analysis of a collaborative web-based database for lupus erythematosus-associated skin lesions: prospective enrollment of 114 patients

    Archives of Dermatology

    (2009)
  • R.D. Sontheimer et al.

    Subacute cutaneous lupus erythematosus: a cutaneous marker for a distinct lupus erythematosus subset

    Archives of Dermatology

    (1979)
  • M. Caproni et al.

    Subacute cutaneous lupus erythematosus with pityriasis-like cutaneous manifestations

    International Journal of Dermatology

    (2001)
  • A. Kuhn et al.

    Clinical manifestations of cutaneous lupus erythematosus

    Journal der Deutschen Dermatologischen Gesellschaft

    (2007)
  • M.R. Cohen et al.

    Systemic disease in subacute cutaneous lupus erythematosus: a controlled comparison with systemic lupus erythematosus

    Journal of Rheumatology

    (1994)
  • R.D. Sontheimer et al.

    Serologic and HLA associations in subacute cutaneous lupus erythematosus, a clinical subset of lupus erythematosus

    Annals of Internal Medicine

    (1982)
  • C.M. Gronhagen et al.

    Subacute cutaneous lupus erythematosus and its association with drugs: a population-based matched case-control study of 234 patients in Sweden

    British Journal of Dermatology

    (2012)

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