Introduction
Discoid Lupus Erythematosus (DLE) is a chronic, benign inflammatory skin disorder. It predominantly affects sun-exposed areas, particularly the face and scalp, and is characterized by well-demarcated, erythematous, scaly plaques that typically heal with atrophy, dyspigmentation, and scarring1. These features can lead to permanent cosmetic disfigurement, especially when diagnosis and treatment are delayed2. Among the types of cutaneous lupus, DLE is both the most common and the most disfiguring form of chronic cutaneous lupus erythematosus3.
Epidemiologically, DLE accounts for approximately 80% of all cases of cutaneous lupus erythematosus. A Scandinavian population-based study reported an incidence of 4 per 100,000 individuals annually, a finding echoed by American studies. The disease is more prevalent in young to middle-aged adults, with a female-to-male ratio of roughly 2:1. It also shows higher prevalence among individuals of Asian, African, Afro-Caribbean, and Hispanic descent 1,4.
Chronic DLE lesions are prone to permanent scarring, and cicatrial alopecia. It also commonly progresses to dyspigmentation of skin. Although rare, squamous cell carcinoma has been reported to arise in long-standing lesions 5. Furthermore, while classic DLE progresses to systemic lupus erythematosus (SLE) in only about 5% of cases, generalized DLE carries a significantly higher risk.
Treatment can effectively reduce active inflammation, but residual skin damage often remains. DLE lesions characteristically leave atrophic central scarring, telangiectasia, hypopigmentation, and progressively enlarge, leading to increase disfigurement. The treatment of DLE can effectively reduce active inflammation, but residual disfigurement often remains. These findings highlight the critical importance of early diagnosis and management to prevent long-term consequences, including cosmetic disfigurement, pigmentary changes, and potential systemic involvement of SLE 2.
Case report
A 69-year-old male presented with a decade-long history of non-healing, reddish lesions on the scalp, with new facial lesions. The patient has a history of prolonged sun exposure during the time he lived in the rural area where he never used sun protective measures.
The lesions appeared without any prior trauma and were accompanied by mild itching and a burning sensation. These symptoms worsened following sun exposure, resulting in increased erythema. The patient reported that progressive enlargement of the lesions over time and localized hair loss on the scalp. He denied any systemic symptoms such as fever, malaise, or joint pain.
His other medical history was unremarkable, with no family history of autoimmune or other dermatological disorders. He had been treated previously with topical and systemic antifungal and antibiotic medications, all of which were ineffective.
On systemic examination, no significant systemic findings were noted. On dermatological examination, there were erythematous plaques with well-defined borders, primarily located on the scalp and left side of the face as well as hair loss on the scalp lesion.
Those plaques had adherent scales and central atrophy. but no evidence of the Auspitz sign. Regional lymph nodes were not palpable. The examination of the lips and oral mucosa showed no abnormalities.
Laboratory investigations such as a complete blood count, erythrocyte sedimentation rate, liver function test, and renal function test, were within normal limits and ANA and ENA profiles were negative. The provisional diagnosis of this case is cutaneous lupus erythematosus (localized Discoid Lupus Erythematosus).
Treatment began with oral hydroxychloroquine (200 mg at bedtime) and methylprednisolone (8 mg per day) for 2 months. Effective sun protection was advised, along with the application of topical steroids and tacrolimus 0.1% ointment. After two months, there was significant improvement in erythema and size. However, hypopigmentation, and central atrophic scarring on face and scarring alopecia on scalp remained.
Fig.1. Involvement of scalp with residual erythema, central atrophy and scarring alopecia with sharply marginated border.
Fig.2. Examination of the left side of face shows hypopigmented scarred plaque with well defined, slightly hyperpigmented borders and active erythema within the plaques.
Discussion
This case describes a middle-aged male presenting with chronic progressive skin lesions localized on sun- exposed area, especially the scalp, leading to scarring alopecia. The patient also had rural lifestyle and prolonged sun exposure without consistent use of sun protection.
Discoid Lupus Erythematosus is chronic, inflammatory dermatosis predominantly affecting sun-exposed areas, particularly the face, scalp, and ears. Clinically, DLE lesions initially appear as erythematous macules with adherent scale, progressing to atrophy, pigmentary changes, and scarring. DLE lesions initially appear as erythematous macules with adherent scales and then develop into lesions with scarring, atrophy and pigment changes 4. These features were observed in this patient, along with scarring alopecia.
There are two clinical variants: localized DLE, which is confined to the head and neck, and disseminated DLE, which involves areas below the neck, including the trunk and extremities. The disseminated form is not only more clinically aggressive but is also more strongly associated with systemic lupus erythematosus 4. This case is localized DLE because the lesions are only on the scalp and face.
Dutz and Khosravi-Hafshejani reported that immunoglobulins and completements are normal in patients with DLE 1, and Sontheimer, et al (2019), revealed that ANA are present in low titer in 30 to 40 % of DLE patients 2. Moreover, a study of DLE in Turkey reported that ANA positivity was found in male 15.2 % and female 32.3 % of DLE patients 6.
In this localized DLE case, there were no systemic symptoms, and laboratory investigations (including negative ANA and ENA profiles) were unremarkable. This patient initially managed with antibiotic and antifungal therapies, the lesions progress over time and led to scarring and disfigurement, causing distress. Panjwani, 2010 also stated that DLE has been shown to significantly affect quality of life, particularly due to visible disfigurement and psychosocial distress 7.
Although hydroxychloroquine, corticosteroids, topical tacrolimus, and sun avoidance were employed, the patient developed irreversible complications, including scarring alopecia and hypopigmented, atrophic patches.
According to literature, disseminated DLE is more clinically aggressive, may be treatment- recalcitrant, and carries a greater risk of SLE. While localized DLE, although generally confined to the skin if not diagnosed and treated timely and it can still result in significant risk of disfigurement, particularly on the scalp and face 4.
In accordance with the literature, in our case as well, despite appropriate therapy with hydroxychloroquine, corticosteroids, tacrolimus, and sun avoidance, the patient still has irreversible complications, including scarring alopecia and hypopigmented atrophic patches. These outcomes underscore the importance of early recognition, accurate diagnosis, and prompt intervention to limit long-term damage.
Though, CLE is less severe course and a better prognosis, it may lead to SLE, and to severe work-related disability and limited life quality. Therefore, early diagnosis of patients with CLE who are at risk for SLE development and preventive measures against disease-triggering factors are crucial for patients with CLE 8.
This case highlights the critical importance of early clinical awareness and prompt treatment of DLE to prevent irreversible disfigurement and optimize patient outcomes through timely intervention and education.
Conclusion
“Early recognition is essential in DLE patients who have chronic, photosensitive, and scarring skin lesions —even when systemic signs are absent. While treatment can control disease progression, permanent disfigurement may remain, emphasizing the importance of timely intervention, continuous monitoring, and comprehensive patient counseling.”
References
- Dutz, J. and Khosravi-Hafshejani, T. (2024) ‘Lupus Erythematosus and Antiphospholipid Syndrome’in Griffiths, C., Barker, J., Bleider,T., Hussain, W. and Simpson, R. (eds) Rook’s textbook of Dermatology, Tenth Edition. London: John Wiley and Sons Ltd. Pp.51.1-51.45.
- Sontheimer, C.J., Costner, M.I. and Sontheimer, R.D., (2019) ‘Lupus Erythematosus’ in Kang et al. (eds) Fitzpatrick’s Dermatology. Ninth edition. London: McGraw-Hill Education. pp. 1037-1058.
- Quiroga, J.C., Garcia, S.A., Mate, A.R., (2019) ‘ Current Insights Into The Management Of Discoid Lupus Erythematosus’ Clin Cosmet Investig Dermatol pp.721-732.
- James, W.D., Elston, D.M., Treat, J.R., Rosenbach, M.A. and Neuhaus, I.M. (2020) ‘Connective Tissue Diseases’ in Andrew’s disease of the skin, Clinical Dermatology. Thirteenth edition. United States: Elsevier Ltd. pp. 157-166.
- Lee, L.A. and Werth, V.P. (2024) ‘Lupus Erythematosus’ in Bolognia, J.L., Schaffer, J.V. and Cerroni, L. (eds) Dermatology, Fourth edition. United States: Elsevier Ltd. pp. 670-687.
- Yavuz, G.O., Yavuz, I.H., Bayram, I., Aktar, R., Bilgili, S.G. (2019) ‘Clinic experience in discoid lupus erythematosus: a retrospective study of 132 cases’. Postepy Dermatol Alergol. 36(6), pp.739-743.
- Panjwani, S.(2009)’ Early diagnosis and treatment of discoid lupus erythematosus’. J Am Board Fam Med.22(2), pp206-213.
- Durosaro, O., Davis, M.D.P., Reed, K.B., Rohlinger, A. (2009). ‘Incidence of cutaneous lupus erythematosus, 1965-2005: a population-based study’ Arch Dermatol. 145(3), pp.249-253.
Author Information
Myat-Sanda-Kyaw1, Su-Myat-Hlaing 2, Thuya-Aung 3, Mie-Mie-Kyaw4, Thuzar-Aung5
- Professor and Head of Department of Dermatology, UM-2, NOGH
- Lecturer in Department of Dermatology, UM-2, NOGH
- Assistant Lecturer in Department of Dermatology, U M-2
- Specialist Assistant Surgeon, Department of Dermatology, NOGH
- Specialist Assistant Surgeon, Department of Dermatology, NOGH
