Core Dermatology Diagnoses / CategoriesInflammatory

Discoid lupus erythematosus

Author: Faculty Reviewer: Resident Reviewer:

Publish date: Posted on
Last updated: May 31, 2022

Keywords #

discoid lupus erythematosus
connective tissue disease
autoimmune

Diagnosis #

Discoid lupus erythematosus (DLE) is the most common form of chronic cutaneous lupus erythematosus (CCLE). The condition is autoimmune in nature and primarily affects the neck, face, and scalp of young and middle-aged females. DLE manifests initially as discrete, circular, erythematous plaques with a white adherent scale, ultimately progressing to scars, atrophy, and dyspigmentation. There are two forms of DLE: localized, where lesions are limited to sites above the neck, and generalized, where lesions can occur above and/or below the neck.

While the scars seen in DLE can be disfiguring and psychosocially disabling for patients, the condition’s prognosis is good with only 5-28% of patients developing systemic lupus erythematous (SLE).[1] Those with generalized DLE are at an increased risk of SLE (15-28%), while only 5-10% of localized DLE patients develop SLE.[1] Despite this, a thorough history and screening labs should be performed to screen patients for SLE disease activity and damage.

Squamous cell carcinoma can develop in sites of DLE due to chronic inflammation, occurring in 2-3% of DLE patients.[12] Extreme heat, cold, trauma, and smoking have been shown to worsen the condition.[13, 14] The disease course of DLE is chronic, but with adequate and early therapy initiation, it is usually controllable.

Key Concepts #
  • Discoid lupus erythematosus is a scarring condition.
  • Only 5-28% of patients with discoid lupus erythematosus develop systemic lupus erythematosus but all patients should be screened for SLE.
  • The condition manifests as asymptomatic erythematous plaques with white adherent scale, ultimately progressing to atrophic scars most frequently on the neck, face, and scalp.
  • Biopsy is diagnostic and demonstrates hyperkeratosis, follicular plugging, basal layer vacuolar degeneration, and mononuclear cell infiltrate.
  • Squamous cell carcinomas can develop in sites of discoid lupus erythematosus.
  • Sun protection, topical corticosteroids, intralesional corticosteroids, and systemic anti-malarial medications are the mainstays of therapy.
  • Early intervention is essential to prevent formation of new scars.
Epidemiology #

DLE is an uncommon condition that affects predominantly women in the third to fourth decades of life. Individuals of Hispanic or African American descent are at an increased risk of developing DLE. While DLE is the most common form of CCLE, accounting for 73-80% of CCLE cases, the exact incidence of DLE is not known.[2,3]

Clinical Features #
  • Initially, DLE appears clinically as discrete, disc-shaped, slightly indurated purple-red plaques covered by white adherent scale.
  • Older lesions appear as depressed, atrophic scars with associated telangiectasias and dyspigmentation.
  • Often, the scars exhibit central hypopigmentation with a peripheral rim of hyperpigmentation, particularly in those with darker skin phenotypes.
  • The lesions commonly involve sun-exposed areas including the neck, face, scalp and arms.
  • Regions above the neck are affected in the more common, localized form of the disease, whereas the generalized form can also affect the torso and upper extremities.
  • Involvement of the scalp frequently leads to scarring alopecia.
  • DLE can also involve the nails and mucosal surfaces, commonly involving the palate.
  • The white adherent scale observed in DLE accumulates as the lesions mature. Removing the scale of DLE lesions reveals an underside with spiny, follicle-based keratotic spikes, termed the “carpet tack” sign. These keratotic spikes resemble the follicular plugs of dilated hair follicles observed in DLE.
  • Hyperkeratotic DLE is a variant of DLE that is characterized by hyperkeratotic, verrucous plaques.[4]
  • Palmoplantar DLE is another rare variant of DLE resulting in painful, often disabling, erosive lesions on acral surfaces.[5]
Differential Diagnoses #
Diagnostic Workup #

Diagnosis is confirmed through skin biopsy and histopathologic examination. Biopsy demonstrates interface dermatitis, hyperkeratosis, follicular plugging, basal layer vacuolar degeneration, basement membrane thickening, and perivascular and periadnexal mononuclear cell infiltrate (predominantly T cells).[6] Direct immunofluorescence may reveal immunoglobulins and complement localized to the dermoepidermal junction, termed the ‘lupus band’. Use of the lupus band test is limited by false-positive results on sun-exposed skin, and by false-negative results in chronic lesions.[7]

In addition to a detailed history, physical exam, and biopsy, patients with DLE should be screened for SLE with a detailed history, including questions focusing on SLE symptoms—hair loss, arthritis, oral or nasal involvement, and Raynaud’s phenomenon. Lab screening, including complete blood cell count with differential, urinalysis, erythrocyte sedimentation rate, chemistry panel, and an antinuclear antibodies (ANA) test should also be done on all patients with DLE.

Treatment #

Treatment goals in DLE are to improve the appearance of existing scars, and to prevent new lesions/scars through early intervention. Management is focused on sun-protective practices/photoprotection and topical therapies, with systemic therapies reserved for generalized or refractory cases. Sunscreens covering both UVA and UVB radiation are recommended, as well as sun-protective clothing and sun avoidance. High-potency topical corticosteroids (class 1-2) can be of benefit but typically are not fully effective. Having patients use a medium- or high-potency topical corticosteroid daily for two weeks followed by replacement with a nonsteroidal topical calcineurin inhibitor, such as pimecrolimus cream or tacrolimus ointment, daily for two weeks and repeating this routine in a cycling fashion can minimize the risk of topical corticosteroid atrophy. Concerns about the risk of steroid atrophy are mitigated somewhat when treating a potentially scarring skin disorder such as discoid LE. Monthly intralesional triamcinolone may also be utilized in active lesions. While topical calcineurin inhibitors do not cause atrophy, their efficacy when used alone in managing DLE lesions is limited.[8]

Oral antimalarials—including hydroxychloroquine, chloroquine, and quinacrine —can be employed in cases of extensive DLE or those recalcitrant to topical or local therapies.[9] Patients on antimalarial therapy should undergo yearly ophthalmologic examination to monitor for retinal toxicity.[10] Smoking may reduce the effectiveness of antimalarial therapy in DLE.[11] Alternative systemic treatments include retinoids (isotretinoin or acitretin), dapsone, thalidomide, azathioprine, mycophenolate mofetil, and methotrexate.[9]

References #
  1. Chong BF, Song J , Olsen NJ. Determining risk factors for developing systemic lupus erythematosus in patients with discoid lupus erythematosus. The British journal of dermatology 2012;166:29-35.
  2. Cardinali C, Caproni M, Bernacchi E, Amato L , Fabbri P. The spectrum of cutaneous manifestations in lupus erythematosus–the Italian experience. Lupus 2000;9:417-23.
  3. Durosaro O, Davis MD, Reed KB , Rohlinger AL. Incidence of cutaneous lupus erythematosus, 1965-2005: a population-based study. Archives of dermatology 2009;145:249-53.
  4. Farley-Loftus R, Elmariah SB, Ralston J, Kamino H , Franks AG, Jr. Hypertrophic discoid lupus erythematosus. Dermatology online journal 2010;16:1.
  5. Prystowsky SD, Herndon JH, Jr. , Gilliam JN. Chronic cutaneous lupus erythematosus (DLE)–a clinical and laboratory investigation of 80 patients. Medicine (Baltimore) 1976;55:183-91.
  6. Patel P , Werth V. Cutaneous lupus erythematosus: a review. Dermatologic clinics 2002;20:373-85, v.
  7. Fabre VC, Lear S, Reichlin M, Hodge SJ , Callen JP. Twenty percent of biopsy specimens from sun-exposed skin of normal young adults demonstrate positive immunofluorescence. Archives of dermatology 1991;127:1006-11.
  8. Kuhn A, Gensch K, Haust M, Schneider SW, Bonsmann G, Gaebelein-Wissing N et al. Efficacy of tacrolimus 0.1% ointment in cutaneous lupus erythematosus: a multicenter, randomized, double-blind, vehicle-controlled trial. Journal of the American Academy of Dermatology 2011;65:54-64, .e1-2.
  9. Chang AY , Werth VP. Treatment of cutaneous lupus. Curr Rheumatol Rep 2011;13:300-7.
  10. Marmor MF, Kellner U, Lai TY, Lyons JS , Mieler WF. Revised recommendations on screening for chloroquine and hydroxychloroquine retinopathy. Ophthalmology 2011;118:415-22.
  11. Jewell ML , McCauliffe DP. Patients with cutaneous lupus erythematosus who smoke are less responsive to antimalarial treatment. Journal of the American Academy of Dermatology 2000;42:983-7. /li>
  12. Zaalberg A, Moradi Tuchayi S, Ameri AH, Ngo KH, Cunningham TJ, Eliane JP et al. Chronic Inflammation Promotes Skin Carcinogenesis in Cancer-Prone Discoid Lupus Erythematosus. The Journal of investigative dermatology 2019;139:62-70.
  13. Miot HA, Bartoli Miot LD , Haddad GR. Association between discoid lupus erythematosus and cigarette smoking. Dermatology (Basel, Switzerland) 2005;211:118-22.
  14. Berger E, Robinson M, Patel R , Franks AG, Jr. Koebner phenomenon to heat in cutaneous (discoid) lupus erythematosus (lupus ab-igne). Dermatology online journal 2012;18:17.
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