Discoid lupus erythematosus (DLE) is the most common form of chronic cutaneous lupus erythematosus (CCLE) and occurs as localised form (ca. 80%) or disseminated/generalized form (ca. 20%). The localised form presents with lesions on the face and scalp, especially the cheeks, forehead, ears, nose, and upper lip, whereas the generalized form presents with lesions involving the upper part of the trunk and the extensor aspects of the extremities.¹ The lesions of DLE consist of sharply-demarcated, coin-shaped (‘discoid’) indurated erythematous plaques with adherent follicular hyperkeratosis.² During the course of the disease the lesions may expand at the periphery with an active erythematous border and hyperpigmentation, resulting in atrophy, scarring, telangiectasia and hypopigmentation in the center of the lesions. At the scalp, eyebrows and bearded regions of the face, DLE can progress to total, irreversible scarring alopecia. In the perioral region, the lesions can lead to characteristic pitted acneiform (‘vermicular’) scarring.³ Mucosal DLE presents with chronic buccal plaques, showing typical roundish lesions with peripheral white hyperkeratotic striae and central atrophy, erosion or ulceration. Exposure to the sun or irritating stimuli (‘Koebner phenomenon’), such as trauma, can provoke or exacerbate the disease.⁴ DLE lesions occur in approximately 15–25% of patients in the course of SLE, but more than 95% of patients with DLE lesions suffer from cutaneous disease only. First-line treatment options in DLE include topical corticosteroids or calcineurin inhibitors; in patients with disfiguring and widespread disease, systemic agents need to be applied.⁵ The first-line systemic treatment is antimalarials, but some patients are therapy-resistant and immunosuppressive agents, such as methotrexate or mycophenolate mofetil, are used as alternative therapeutic option. The monoclonal antibody belimumab, which is approved for SLE as an adjunct therapy for patients with autoantibody-positive disease who despite standard therapy show high disease activity, may be effective, but needs to be evaluated using validated skin scores.

Learning objectives

• Describe the different types of skin manifestations in DLE

• Explain the preventive strategies for DLE including photoprotection

• Discuss the topical and systemic treatment options for DLE

References

1. Costner M, Sontheimer R, Provost T. Lupus erythematosus. In: Sontheimer R, Provost T, eds. Cutaneous manifestations of rheumatic diseases. Philadelphia: Williams & Wilkins, 2003.

2. Kuhn A, Landmann A, Bonsmann G. Cutaneous lupus erythematosus. In: GC T, ed. Systemic Lupus Erythematosus. 1st ed. Amsterdam: Systemic Lupus Erythematosus, 2016:333–40.

3. Chang YH, Wang SH, Chi CC. Discoid lupus erythematosus presenting as acneiform pitting scars. International journal of dermatology 2006;45(8):944–5.

4. Ueki H. Koebner phenomenon in lupus erythematosus with special consideration of clinical findings. Autoimmun Rev 2005;4(4):219–23.

5. Kuhn A, Aberer E, Bata-Csorgo Z, et al. S2k guideline for treatment of cutaneous lupus erythematosus - guided by the European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV). Journal of the European Academy of Dermatology and Venereology : JEADV 2017;31(3):389–404.