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Lupus Erythematosus, Discoid
Updated: Apr 14, 2009
Introduction
Background
Discoid lupus erythematosus (DLE) is a chronic, scarring, atrophy producing, photosensitive dermatosis. Discoid lupus erythematosus may occur in patients with systemic lupus erythematosus (SLE), and some patients (<5%) with discoid lupus erythematosus progress to SLE.1 Some patients also have the lesions of subacute cutaneous lupus erythematosus (SCLE), and some may have a malar rash. Patients with discoid lupus erythematosus rarely fulfill 4 or more of the criteria used to classify SLE. Serologic abnormalities are uncommon. Therapy with sunscreens, topical corticosteroids, and antimalarial agents is usually effective.
Pathophysiology
Discoid lupus erythematosus (DLE) probably occurs in genetically predisposed individuals, but the exact genetic connection has not been determined. The pathophysiology of discoid lupus erythematosus is not well understood. It has been suggested that a heat shock protein is induced in the keratinocyte following ultraviolet (UV) light exposure or stress, and this protein may act as a target for gamma (delta) T-cell–mediated epidermal cell cytotoxicity.2
Frequency
United States
Worldwide, the prevalence of SLE ranges from 17-48 cases per 100,000 population. The highest prevalence of SLE occurs in persons aged 40-60 years, and is approximately 10 times higher in women than in men. Cutaneous lupus erythematosus (CLE) presumably occurs 2-3 times more frequently in women than in men. Discoid lupus erythematosus is responsible for 50-85% of CLE patients.
Mortality/Morbidity
Patients with discoid lupus erythematosus rarely have clinically significant systemic disease. Lesions may produce scarring or atrophy. Scarring alopecia is particularly disturbing.
Race
Discoid lupus erythematosus is slightly more common in African Americans than in whites or Asians.
Sex
The male-to-female ratio of discoid lupus erythematosus is 1:2.
Age
Discoid lupus erythematosus may occur at any age but most often occurs in persons aged 20-40 years. The mean age is approximately 38 years.
Clinical
History
- Patients may complain of mild pruritus or occasional pain within the lesions, but most patients are asymptomatic.
- Approximately 5% or less discoid lupus erythematosus (DLE) patients have accompanying systemic involvement.
- Arthralgia or arthritis may occur.
- Patients may manifest any symptom of SLE; therefore, the history should include an assessment for symptoms of pleuritis, pericarditis, neurologic involvement, and renal involvement.
- Several cutaneous diseases have been reported, perhaps in greater frequency, in patients with discoid lupus erythematosus.
- Malignant degeneration of chronic lesions of lupus erythematosus (LE) is possible, although rare, leading to nonmelanoma skin cancer. Dark-skinned individuals may be more prone to skin cancer because of the lack of pigmentation within the chronic lesion, combined with chronic inflammation and continued sun damage.
- Mucin deposition is a factor in the histopathology of LE. Some patients develop such a massive amount of mucin that lesions become raised and assume a different morphology.
- Porphyria cutanea tarda appears to be overrepresented in LE patients. Often, the porphyria is discovered when antimalarials first are administered.
- Lichen planuslike lesions may be part of an overlap between LE and lichen planus or may occur as a result of antimalarial therapy.
- Psoriasis is a common disease, although it is not clear whether it is more common in LE patients.
Physical
- Discoid lupus erythematosus lesions (DLE) frequently are characteristic. The primary lesion is an erythematous papule or plaque with slight-to-moderate scaling (Media Files 1-2). As the lesion progresses, the scale may thicken and become adherent, and pigmentary changes may develop, with hypopigmentation in the central or inactive area and hyperpigmentation at the active border.
Discoid lupus erythematosus on the face.
Chronic scarred lesion of discoid lupus erythematosus.
- Lesions spread centrifugally and may merge. As lesions age, dilation of follicular openings occurs with a keratinous plug, termed follicular plugging or patulous follicles (Media File 3). Resolution of the active lesion results in atrophy and scarring.
Lesions of discoid lupus erythematosus in the conchal bowl demonstrate patulous follicles with follicular plugging.
- At any time, individual lesions may have any or all of these features. Early lesions may be difficult to distinguish from SCLE. Discoid lupus erythematosus lesions often are photodistributed, but relatively unexposed skin also may be affected. The scalp is a common area of involvement, and permanent alopecia may result (Media Files 5-6).
Scarring alopecia of discoid lupus erythematosus.
Widespread scarring alopecia.
- Patients with discoid lupus erythematosus often are divided into 2 subsets: localized and widespread. Localized discoid lupus erythematosus occurs when the head and neck only are affected, while widespread discoid lupus erythematosus occurs when other areas are affected, regardless of whether disease of the head and neck is seen (Media File 7). Patients with widespread involvement often have hematologic and serologic abnormalities, are more likely to develop SLE, and are more difficult to treat.
Truncal lesions of chronic cutaneous lupus erythematosus.
- Several unusual variants of chronic CLE, other than discoid lupus erythematosus, have been reported.
- Mucosal surfaces may be affected by lesions that appear identical to discoid lupus erythematosus of the skin or by lesions that may simulate lichen planus.
- Palms and soles may be affected, but this occurs in less than 2% of patients (Media File 4).3
Palmar lesions of discoid lupus erythematosus.
- Discoid lupus erythematosus lesions may become hypertrophic or verrucous (Media File 8). This subset is manifested by wartlike lesions, most often on the extensor arms. Hypertrophic lesions of LE must be differentiated from warts, keratoacanthomas, or squamous cell carcinoma. These lesions are more difficult to treat.4
Hypertrophic lesions of lupus erythematosus on the dorsal hands. Characteristic lesions were observed elsewhere.
- Lupus panniculitis is a form of chronic CLE that may be accompanied by typical DLE lesions or may occur in patients with SLE.5
Causes
- Patients with discoid lupus erythematosus probably have genetic predisposition; however, the precise genetic factors that increase the risk of this disease are unknown. The disease usually manifests following UV light exposure, but other triggers or inciting factors also must contribute. Toll-like receptors are possibly involved in the pathogenesis.
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References
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Further Reading
Keywords
chronic cutaneous lupus erythematosus, discoid lupus erythematosus
