Lupus erythematosus

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A. The lupus band test (LBT) demonstrates deposits of immunoglobulin and complement at the dermal-epidermal junction by direct immunofluorescent staining of the skin. Biopsy of cutaneous lesions in DLE or systemic lupus erythematosus (SLE) yields a positive LBT in about 90% of patients.

Clinically normal skin of patients with DLE demonstrates a negative LBT. The LBT in clinically normal skin of patients with SLE varies with sun exposure. Approximately 50% of patients with SLE have a positive LBT in clinically normal, sun-protected skin, whereas 80% have a positive LBT in clinically normal, sun-exposed areas.

B. Discoid lupus erythematosus. The characteristic lesion of DLE is a scaly plaque that ranges in color from red to violaceous, with sharply-defined borders, central atrophy, telangiectasia, and areas of hypopigmentation or hyperpigmentation. Keratinous plugging of the hair follicles can sometimes be detected as tiny rough projections across the lesion. Lesions may be multiple and asymmetric and are most commonly found on the head and neck, particularly in the malar areas, ears, and scalp. When the scalp is involved, hair loss with scarring at the site of the lesion results. Lesions on the oral and nasal mucosae may ulcerate. Lesions of DLE produce scarring; those that result in severe scarring may, infrequently, produce skin cancer.

When discoid lesions are present below the neck, the term generalized DLE is used. SLE develops in fewer than 10% of patients who present with lesions of DLE.

C. Systemic lupus erythematosus. Cutaneous manifestations of SLE comprise several of the criteria for the classification of SLE. These criteria are facial erythema, DLE lesions, photosensitivity, and oral or nasopharyngeal ulceration.

1. Facial erythema. The classic butterfly rash of SLE occurs in up to 40% of patients. It begins as a transient erythematous, edematous eruption across the bridge of the nose and malar areas. It is often exacerbated by sun exposure and may accompany a flare of systemic disease. If the rash is persistent, atrophy, telangiectasia, and scaling will develop.

2. Discoid lesions seen in SLE are identical to those of DLE and occur in approximately 20% to 30% of patients. Patients with DLE only above the neck are at less risk for development of SLE.

3. Photosensitivity will often precipitate butterfly-pattern erythema, the rash of subacute lupus erythematosus, and lesions of DLE. The active spectrum is ultraviolet light of 280 to 320 nm (ultraviolet b), which normally produces sunburn erythema. Ultraviolet A (320 to 400 nm) has been implicated in lesions of subacute cutaneous lupus erythematosus (SCLE). Sunlight may also cause a flare of systemic disease.

4. Oral or nasopharyngeal ulcerations are shallow and have gray bases with red borders. They are often painful and usually seen in patients with severe cutaneous disease.

5. Raynaud's phenomenon is seen in up to 30% of cases.

6. Alopecia can be of two types in SLE.

a. Scarring alopecia is produced when discoid lesions affect the scalp; these lesions are easily recognized.

b. More subtle is the reversible (diffuse or patchy) alopecia that may arise. Diffuse alopecia is more common than patchy alopecia. It may accompany a clinical flare of SLE and is sometimes elicited only by specifically questioning the patient about increased hair loss. The frontal hairline may consist of short, broken hairs, as in traction alopecia.

7. Subacute cutaneous lupus erythematosus is a nonscarring photosensitive eruption with papulosquamous and annular-polycyclic patterns. The distribution is over sun-exposed areas. Patients have a high frequency of anti-Ro auto-antibodies and generally have a milder systemic disease.

a. The papulosquamous eruption is composed of confluent and discrete scaly erythematous papules and plaques.

b. In the annular-polycyclic type, scaly erythematous borders surround central areas of subtle hypopigmentation and telangiectasia.

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