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Discoid Lupus Erythematosus

Original Editor - Ahmed M Diab
Top Contributors - Ahmed M Diab, Vidya Acharya and Alexandra Stead

Introduction

Discoid lupus erythematosus (DLE) represents the most prevalent subtype of chronic cutaneous lupus erythematosus (CCLE), accounting for nearly 62–83% of all chronic lupus erythematosus patients. [1]DLE is characterised by persistent, inflammatory skin lesions that often lead to scarring, scaling plaques and atrophy. It primarily affects sun-exposed areas such as the face, scalp and ears. [2][3] [4]The disease is classified as localized (above the neck) or generalized/disseminated (above and below the neck). Generalized DLE "typically involves the extensor forearms and hands" and may involve the anterior chest and upper back. [3]The disseminated form of DLE, especially when involving the trunk, is associated with an increased risk of progression to Systemic Lupus Erythematosus (SLE). [2] While DLE is generally confined to the skin in 80-90% of cases, it carries a risk of progression to systemic lupus erythematosus (SLE) in up to 28% of generalized presentations. [5] As a chronic autoimmune condition, DLE imposes significant psychosocial burdens due to disfigurement, particularly in patients with darker skin tones where post-inflammatory hyper- or hypopigmentation is pronounced.[4]Female patients comprised 73% of the overall DLE population, which corresponds to a female:male ratio of approximately 2.7:1. [5] Given that Discoid Lupus Erythematosus (DLE) is associated with significant fatigue and arthralgias, and carries a risk of progression to Systemic Lupus Erythematosus (SLE) (a condition that typically affects the joints), it is essential that physiotherapists understand DLE to conduct a comprehensive functional assessment and implement holistic rehabilitation interventions for these patients. [2] [6]

Discoid lupus erythematosus is the most common form of chronic cutaneous lupus, affecting 62-83% of chronic lupus patients. It causes persistent, inflammatory skin lesions that result in scarring, scaling, and atrophy, primarily on sun-exposed areas like the face, scalp, and ears.DLE is classified as either localized (above the neck) or generalized/disseminated (above and below the neck, including forearms, hands, chest, and upper back). While 80-90% of cases remain confined to the skin, the generalized form—particularly when involving the trunk—carries up to a 28% risk of progressing to systemic lupus erythematosus.The condition predominantly affects females, with a 2.7:1 female-to-male ratio. Beyond visible disfigurement, DLE causes significant psychosocial burden, especially in patients with darker skin tones who experience pronounced pigmentation changes. Patients also commonly experience fatigue and joint pain.[1][2][3][4] [5][6]

Pathophysiology

Genetic and Environmental Factors:

DLE is an autoimmune disease caused by a combination of genetic vulnerability and environmental triggers. Certain genetic variations (in genes like TYK2, IRF5, and CTLA4) make some people more susceptible by weakening the immune system's ability to distinguish between the body's own cells and foreign invaders. This genetic susceptibility also increases the production of inflammatory proteins called type I interferons.

Environmental factors can trigger or worsen the disease in genetically susceptible individuals. Two major triggers are:

  • UV radiation (sunlight exposure)
  • Cigarette smoking, which damages skin cells, promotes abnormal immune cell activity, and causes DNA damage that leads the body to produce antibodies against its own DNA

The Immune Response in DLE:

When UV light damages skin cells (keratinocytes), these dying cells release distress signals and genetic material. This activates specialised immune cells called plasmacytoid dendritic cells, which then produce inflammatory proteins (interferons).

These interferons attract aggressive immune cells, particularly cytotoxic CD8+ T cells, which are the dominant cell type found in DLE skin lesions. These cells create a strong inflammatory response characterized by high levels of interferon-gamma and granzyme B (proteins that damage tissue).

Other immune cells also contribute to the disease:

  • B cells present antigens and release inflammatory molecules like IL-6
  • Neutrophils form web-like structures (NETs) that trap pathogens but also amplify inflammation through antimicrobial proteins like LL-37

Under the microscope, DLE lesions show distinctive features:

  • Inflammation around hair follicles and sweat glands
  • Plugged hair follicles
  • Thickened basement membranes (the layer between skin layers)
  • Mucin deposits in the deeper skin layer

These changes lead to the scarring and fibrosis (tissue thickening) that characterise DLE. [7] [2] [8]

Clinical Features

The DLE rash has defined borders, erythematous plaques with adherent hyperkeratotic scale, often exhibiting the ‘’carpet tack sign’’ upon scale removal —protruding keratotic spikes mirroring dermal papillary inflammation. Lesions evolve from active peripheral inflammation to central hypopigmentation, atrophy and telangiectasias, with scarring in 60-80% of cases. Localized DLE (80% of patients) confines to the head and neck, while generalized forms (<20 lesions above and below the neck) heighten SLE risk. Scalp involvement leads to irreversible scarring alopecia, a common cosmetic concern. Mucosal lesions (e.g., on lips or buccal mucosa) occur in 10-20% of cases, and rare variants include hypertrophic (verrucous) or tumid forms. Symptoms include pruritus, pain or burning, exacerbated by sun exposure. Extracutaneous associations, relevant to physiotherapy, encompass arthralgias (in 10-20%) and myalgias, potentially mimicking fibromyalgia or contributing to reduced physical function. A 2024 spectrum analysis of CLE manifestations notes that DLE patients report higher fatigue scores, impacting daily activities and rehabilitation adherence. [9] [10]

Diagnosis

The diagnosis of Discoid Lupus Erythematosus (DLE) is primarily clinical, but it is definitively confirmed using histopathology (skin biopsy) and often supplemented with serological tests to screen for systemic involvement. The most common morphological form observed is the classic discoid plaque form that is well-demarcated, erythematous, coin-shaped with adherent scale and follicular plugging which, when removed, can show the "carpet tack sign'' . They often lead to central atrophy, scarring, and pigmentary changes (hypo- or hyperpigmentation) and common sites are sun-exposed areas, primarily the face, scalp, and ears. [11][12]A skin biopsy is required to confirm the diagnosis, especially in early or uncertain cases, by demonstrating specific microscopic changes. Direct immunofluorescence may be performed on lesional skin to check for immune deposits. [2]

Treatment

Management prioritises photoprotection (broad-spectrum SPF 50+ sunscreen, UV-avoidant clothing) and smoking cessation. Topical therapies form the first line: high-potency corticosteroids (e.g., clobetasol 0.05%) or calcineurin inhibitors (tacrolimus 0.1%) for facial lesions, with intralesional triamcinolone (3-5 mg/mL) for refractory plaques.[13][14]

Physiotherapy Role

The role of physiotherapy is limited in the primary management of Discoid Lupus Erythematosus (DLE); however, the physiotherapist can evaluate patients susceptible to Systemic Lupus Erythematosus (SLE) progression. Furthermore, a study reports that pulsed ultrasound is effective in DLE scar management.[15]

References

  1. ↑ 1.0 1.1 Elmgren J, Nyberg F. Clinical aspects of cutaneous lupus erythematosus. Frontiers in Medicine. 2023 Jan 9;9:984229. https://pmc.ncbi.nlm.nih.gov/articles/PMC9868707/
  2. ↑ 2.0 2.1 2.2 2.3 2.4 2.5 McDaniel B, Sukumaran S, Koritala T, Tanner LS. Discoid lupus erythematosus. https://www.ncbi.nlm.nih.gov/books/NBK493145/
  3. ↑ 3.0 3.1 3.2 Okon LG, Werth VP. Cutaneous lupus erythematosus: diagnosis and treatment. Best practice & research Clinical rheumatology. 2013 Jun 1;27(3):391-404. https://pmc.ncbi.nlm.nih.gov/articles/PMC3927537/
  4. ↑ 4.0 4.1 4.2 Jessop S, Whitelaw DA, Grainge MJ, Jayasekera P. Drugs for discoid lupus erythematosus. Cochrane Database of Systematic Reviews. 2017(5). https://dermnetnz.org/topics/discoid-lupus-erythematosus
  5. ↑ 5.0 5.1 5.2 Alharbi A, Alamri O, Afandi A, Arbaeen A, Mirza A, Alahmari A, Alshomrani S, Qashqari M, Alahmadi M, Alharthy S, Kokandi A. Discoid lupus erythematosus and its progression to systemic lupus erythematosus across age groups: a systematic review. Journal of Medicine and Life. 2025 Sep;18(9):830. https://pmc.ncbi.nlm.nih.gov/articles/PMC12577789/
  6. ↑ 6.0 6.1 MOHAMED MK, SHERIF EB, MOHAMED TR, MARWA A. Effect of Pulsed Ultrasound on Discoid Lupus Erythematosus. The Medical Journal of Cairo University. 2019 Mar 1;87(March):889-95. https://mjcu.journals.ekb.eg/article_52710.html
  7. ↑ Niebel D, de Vos L, Fetter T, Brägelmann C, Wenzel J. Cutaneous lupus erythematosus: an update on pathogenesis and future therapeutic directions. American Journal of Clinical Dermatology. 2023 Jul;24(4):521-40. https://pubmed.ncbi.nlm.nih.gov/37140884/
  8. ↑ Harris, F., Heuer, S., Rob, R., Bachali, P., Labonte, A., Chong, B., Catalina, M., Lipsky, P. and Grammer, A., 2019, October. Analysis of Discoid Lupus Erythematosus (DLE) Gene Expression Reveals Dysregulation of Pathogenic Pathways Associated with Infiltrating Immune/Inflammatory Cells. In ARTHRITIS & RHEUMATOLOGY (Vol. 71). 111 RIVER ST, HOBOKEN 07030-5774, NJ USA: WILEY.https://acrabstracts.org/abstract/analysis-of-discoid-lupus-erythematosus-dle-gene-expression-reveals-dysregulation-of-pathogenic-pathways-associated-with-infiltrating-immune-inflammatory-cells/
  9. ↑ Fijałkowska A, Kądziela M, Żebrowska A. The spectrum of cutaneous manifestations in lupus erythematosus: a comprehensive review. Journal of Clinical Medicine. 2024 Apr 21;13(8):2419. https://pubmed.ncbi.nlm.nih.gov/38673692/
  10. ↑ Brahmanti H, Wahono CS, Pratama MZ, Rahman PA, Suhendra RB, Rizky AN, Mukti NH. Association of the Spectrum of Cutaneous Lupus Erythematosus with Disease Activity and Systemic Manifestations in Patients with Systemic Lupus Erythematosus. Mediterranean Journal of Rheumatology. 2023 Sep 19;35(1):143. https://pmc.ncbi.nlm.nih.gov/articles/PMC11082774/
  11. ↑ Ashraf E, Ghouse AN, Siddiqui S, Siddiqui S, Khan Z. Discoid lupus erythematosus: a cross-sectional study from the Sindh Institute of skin diseases, Karachi, Pakistan. Cureus. 2020 Oct 27;12(10).https://pmc.ncbi.nlm.nih.gov/articles/PMC7704007/
  12. ↑ Panjwani S. Early diagnosis and treatment of discoid lupus erythematosus. The Journal of the American Board of Family Medicine. 2009 Mar 1;22(2):206-13.https://dermnetnz.org/topics/discoid-lupus-erythematosus
  13. ↑ Company-Quiroga J, Alique-García S, Romero-Maté A. Current insights into the management of discoid lupus erythematosus. Clinical, Cosmetic and Investigational Dermatology. 2019 Oct 3:721-32. https://pmc.ncbi.nlm.nih.gov/articles/PMC6781736/
  14. ↑ Bouché N, Al-Saedy MA, Song EJ. Successful treatment of refractory subacute cutaneous lupus erythematosus with deucravacitinib. JAAD Case Reports. 2023 Sep 1;39:93-5. https://www.jaadcasereports.org/article/S2352-5126(23)00280-1/fulltext
  15. ↑ MOHAMED MK, SHERIF EB, MOHAMED TR, MARWA A. Effect of Pulsed Ultrasound on Discoid Lupus Erythematosus. The Medical Journal of Cairo University. 2019 Mar 1;87(March):889-95. https://mjcu.journals.ekb.eg/article_52710.html
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