Discoid Lupus Erythematosus
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Treatment of almost all medical conditions has been affected by the COVID-19 pandemic. NICE has issued rapid update guidelines in relation to many of these. This guidance is changing frequently. Please visit https://www.nice.org.uk/covid-19 to see if there is temporary guidance issued by NICE in relation to the management of this condition, which may vary from the information given below.
Discoid lupus erythematosus (DLE) is a chronic photosensitive skin eruption which can be either localised or widespread. It is confined to the skin and does not cause any systemic symptoms. DLE can cause permanent scarring if treatment is inadequate.
- The prevalence is between 17 and 48 per 100,000 people.
- Women are much more often affected than men.
- DLE usually presents in people aged between 20 and 40 years of age.
- DLE is more common in smokers.
- DLE can be present in a small proportion of patients with systemic lupus erythematosis (SLE).
- Red scaly patches develop which leave pigmentation, atrophy and white scars.
- The lesions are usually asymptomatic but they may present with mild pruritus or sometimes pain within the lesions.
- DLE mainly affects areas exposed to sunlight, such as the cheeks, nose, ears, upper back, neck and the backs of hands. It may rarely occur on the palms or soles.
- DLE lesions may become hypertrophic, causing wart-like lesions, most often on the extensor aspects of the arms.
- The scalp may be affected and cause permanent scarring alopecia.
- DLE may affect the lips and inside the mouth, causing ulcers and scaling.
- Plaque psoriasis
- Actinic keratosis
- Lichen planus
- Squamous cell carcinoma
- The diagnosis of DLE is usually based on clinical features.
- Biopsy for histology may be required.
- Histopathological changes are characteristic but depend on the type and age of the lesion.
- Most patients with DLE show a direct positive immunofluorescence in biopsies of lesions.
- Serology: approximately 20% of patients with DLE have a positive antinuclear antibody (ANA).
- There may be a low white cell count and raised ESR.
- Complement levels may be low.
- Urinalysis may indicate renal involvement with albuminuria.
Blood tests should be repeated periodically, usually annually when the condition appears stable, to check for the onset of systemic disease.
The mainstay of treatment includes sun protection, topical or intralesional steroids and antimalarial drugs.
- Sun exposure must be minimised by avoiding going out in bright sun as much as possible and by wearing protective clothing and high-factor sunscreens.
- The lesions are unsightly and usually in visible places, so cosmetic camouflage is required.
- Corticosteroids may be used topically or intralesionally:
- Very potent forms are necessary for hypertrophic lesions.
- Systemic steroids do not seem to be effective.
- When systemic treatment is required, hydroxychloroquine is the usually the first-line agent.
- Topical imiquimod cream is an alternative treatment for generalised DLE.
- Other possible treatments include topical retinoids and immunosuppressive agents (eg, azathioprine or methotrexate).
- Burned-out scarred lesions may be excised.
- Photodynamic therapy can be effective for some cases.
- Laser therapy can be considered for lesions with prominent telangiectasias.
- A minority of patients with DLE (fewer than 5%) progress to SLE.
- In paediatric patients, however, DLE carries a significant risk of progression to SLE but may predict a milder phenotype of systemic disease.
- The presence of DLE in patients with SLE has not been shown to be associated with less severe disease.
- There are increased risks of photosensitivity and leukopenia and decreased risks of pleuritis and arthritis in patients with both SLE and DLE.
- Malignant degeneration (basal cell carcinoma or squamous cell carcinoma) may occur but is uncommon.
- Dark skin may lose its inherent protection with depigmentation.
- Pain in lesions may continue and disfiguration from scars and atrophy will be permanent.
- DLE tends to heal with scarring, hair loss and pigmentary changes if treatment is not initiated in the early phase of the disease.
Further reading and references
Bockle BC, Sepp NT; Smoking is highly associated with discoid lupus erythematosus and lupus erythematosus tumidus: analysis of 405 patients. Lupus. 2015 Jun24(7):669-74. doi: 10.1177/0961203314559630. Epub 2014 Nov 19.
Merola JF, Prystowsky SD, Iversen C, et al; Association of discoid lupus erythematosus with other clinical manifestations among patients with systemic lupus erythematosus. J Am Acad Dermatol. 2013 Jul69(1):19-24. doi: 10.1016/j.jaad.2013.02.010. Epub 2013 Mar 28.
Bharti S, Dogra S, Saikia B, et al; Immunofluorescence profile of discoid lupus erythematosus. Indian J Pathol Microbiol. 2015 Oct-Dec58(4):479-82. doi: 10.4103/0377-4929.168850.
Turan E, Sinem Bagci I, Turgut Erdemir A, et al; Successful treatment of generalized discoid lupus erythematosus with imiquimod cream 5%: a case report and review of the literature. Acta Dermatovenerol Croat. 201422(2):150-9.
Debu A, Girard C, Bessis D; Discoid lupus erythematosus successfully treated by photodynamic therapy. Br J Dermatol. 2015 Mar172(3):821-2. doi: 10.1111/bjd.13407. Epub 2015 Jan 18.
Arkin LM, Ansell L, Rademaker A, et al; The natural history of pediatric-onset discoid lupus erythematosus. J Am Acad Dermatol. 2015 Apr72(4):628-33. doi: 10.1016/j.jaad.2014.12.028. Epub 2015 Jan 30.
Saghafi M, Hashemzadeh K, Sedighi S, et al; Evaluation of the incidence of discoid lupus erythematosus in patients with systemic lupus erythematosus and its relationship to disease activity. J Cutan Med Surg. 2014 Oct18(5):316-9.
Fernandes MS, Girisha BS, Viswanathan N, et al; Discoid lupus erythematosus with squamous cell carcinoma: a case report and review of the literature in Indian patients. Lupus. 2015 Dec24(14):1562-6. doi: 10.1177/0961203315599245. Epub 2015 Aug 6.