Discoid Lupus Erythematosus Clinical Presentation

Updated: Aug 21, 2018
  • Author: Elizabeth Tkachenko; Chief Editor: William D James, MD  more...
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Presentation

History

Patients may report mild pruritus or occasional pain within the lesions, but most patients are asymptomatic. According to one study, approximately 16% of patients with discoid lupus erythematosus (DLE) may develop systemic involvement within 3 years of diagnosis. [1] Another cohort study reported that 17% of patients with cutaneous lupus erythematosus (CLE) met criteria for systemic lupus erythematosus (SLE) within a mean time of 8 years. [2] Of note, systemic disease was generally mild in these patients, and the diagnosis of SLE was most commonly established by meeting mucocutaneous and laboratory criteria.

Patients may manifest any sign or symptom of SLE; therefore, the history should include an assessment for serologic and/or hematologic abnormalities, arthralgia or arthritis, pleuritis, pericarditis, neurologic involvement, and renal involvement. A 2013 study demonstrated increased risk of photosensitivity, leukopenia, anti-Smith antibody, and decreased risk of arthritis and pleuritis in patients with SLE and DLE. [16] There was no significant association between DLE and anti-ds DNA antibodies, nephritis, or end-stage renal disease.

Malignant degeneration of chronic lesions of DLE leading to nonmelanoma skin cancer is rare. 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. Reported risk factors for the development of squamous cell carcinoma within lesions of chronic DLE include male sex, early age of onset, refractory disease, lip involvement, and tobacco use. [13]

Porphyria cutanea tarda (PCT) appears to be overrepresented in patients with CLE. Often, PCT is discovered when antimalarials are first administered. Lichen planus–like lesions may be due to an overlap between CLE and lichen planus or may occur as a result of antimalarial therapy.

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Physical Examination

Discoid lupus erythematosus (DLE) lesions frequently are characteristic. The primary lesion is an erythematous papule or plaque with slight-to-moderate scale (see the images below). As the lesion progresses, the scale may thicken and become adherent. Pigmentary changes may develop, with hypopigmentation in the central, or inactive area, and hyperpigmentation at the active border.

Discoid lupus erythematosus on the face. Discoid lupus erythematosus on the face.
Chronic scarred lesion of discoid lupus erythemato 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 (see the image below). Resolution of the active lesion results in atrophy and scarring.

Lesions of discoid lupus erythematosus in the conc 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 those of subacute cutaneous lupus erythematosus (SCLE). DLE lesions are often photodistributed, but relatively unexposed skin may also be affected. The conchal bowls and scalp are both common areas of involvement. Permanent scarring alopecia may result (see images below).

Scarring alopecia of discoid lupus erythematosus. Scarring alopecia of discoid lupus erythematosus.
Widespread scarring alopecia. Widespread scarring alopecia.

Patients with DLE often are divided into two subsets: localized and widespread (disseminated). Localized DLE occurs when the head and neck only are affected, while widespread DLE occurs when other areas are affected, regardless of whether disease of the head and neck is seen. Patients with widespread involvement often have hematologic and serologic abnormalities, are more likely to develop systemic lupus erythematosus (SLE), and are more difficult to treat.

Several unusual variants of chronic cutaneous lupus erythematosus (CCLE), other than DLE, have been reported. Mucosal surfaces may be affected by lesions that appear identical to DLE 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 (see the image below). [17]

Palmar lesions of discoid lupus erythematosus. Palmar lesions of discoid lupus erythematosus.

DLE lesions may become hypertrophic or verrucous (see the image below). This subset is manifested by wartlike lesions, most often on the extensor arms. Hypertrophic lesions of chronic lupus erythematosus must be differentiated from warts, keratoacanthomas, or squamous cell carcinoma. These lesions are more difficult to treat. [18]

Lupus panniculitis, a form of CCLE that manifests with tender, inflammatory nodules in the early phase, results in eventual destruction of the subcutaneous fat with resulting atrophy and contour change. Lupus panniculitis may be accompanied by overlying typical DLE lesions and/or may occur in patients with SLE. [19]

Hypertrophic lesions of chronic cutaneous lupus er Hypertrophic lesions of chronic cutaneous lupus erythematosus on the dorsal hands. Characteristic lesions were observed elsewhere.
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Classification Criteria

Development of classification criteria specific for the diagnosis of DLE was initiated by experts using a Delphi method in 2017. [20] While validation of these criteria is required, they provide a preliminary diagnostic classification scheme to be used clinically and for research. The 12 characteristics, stratified by category, include the following:

Morphology is as follows:

  • Erythematous-violaceous in color
  • Atrophic scarring
  • Dyspigmentation
  • Follicular hyperkeratosis/plugging
  • Scarring alopecia

Histopathology is as follows:

  • Interface/vacuolar dermatitis
  • Perivascular and/or periappendageal lymphohistiocytic infiltrate
  • Follicular keratin plugs
  • Mucin deposition
  • Basement membrane thickening

Location is as follows:

  • Location in conchal bowl
  • Preference for head and neck
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