INTRODUCTION Lupus erythematosus is a chronic inflammatory autoimmune disease with a spectrum of clinical forms ranging from a benign chronic cutaneous variety (discoid lupus erythematosus) to an often-fatal systemic type with nephritis (systemic lupus erythematosus). Intermediate types, variously known as disseminated discoid lupus erythematosus and subacute cutaneous lupus erythematosus, are characterized by various combinations of widespread cutaneous lesions and mild to severe systemic manifestations. The role of immune complexes in the inflammatory manifestations of lupus is well recognized, and in about 4% of cases an associated vasculitis may be seen from small vessel involvement. Lupus erythematosus occurs most commonly in women in the third to fifth decades. When skin lesions occur they typically appear in areas exposed to the ultraviolet rays of the sun. Rarely discoid lupus can degenerate into squamous cell carcinoma.
Common nonscarring eyelid lesions include a pruritic eruption of the lower eyelids. Scarring lesions often present as sharply demarcated purple-red, slightly raised, circumscribed plaques covered with thin adherent whitish scales and telangiectasias. Often such lesions are localized to the lateral aspect of the lower eyelids. Such lesions may enlarge to reach a size of about 5 to 10 mm. The major disfigurement of discoid lupus occurs as the lesions involute where atrophic scarring may lead to trichiasis and entropion. Often, pronounced hypopigmentation or hyper-pigmentation occurs. Other common skin manifestations include the classic butterfly rash, cutaneous vasculitic foci, urticaria, vesiculobullous lesions, and nonscarring alopecia. Ocular manifestations include retinal hemorrhages, cotton wool spots, retinal vasculitis, papillitis, diffuse retinal edema, keratoconjunctivitis sicca, and band keratopathy. Associated systemic findings in lupus erythematosus include arthralgia, nephritis, pleurisy, pericarditis, vasculitis, CNS problems, and hematologic abnormalities.
HISTOPATHOLOGY Active lesions of discoid lupus erythematosus (DLE) exhibit hyperkeratosis, follicular dilation with keratin plugging, epidermal atrophy, hydropic degeneration of the basal layer of epidermis with variable degrees of pigmentary incontinence, basement membrane thickening, occasional apoptotic (cytoid) bodies in the epidermis, dermal edema, mucin deposition in the reticular dermis, and a predominantly perivascular and periappendageal infiltrate of lymphocytes and macrophages. Healing lesions of DLE have hyperkeratosis, atrophic or slightly thickened epidermis, markedly thickened epidermal basement membrane, dermal fibrosis, and marked follicular plugging. Histological features of subacute cutaneous lupus erythematosus and systemic lupus erythematosus overlap and may be indistinguishable from those of DLE.
DIFFERENTIAL DIAGNOSIS The differential diagnosis includes blepharitis, eczema, psoriasis, rosacea, allergic dermatitis, granuloma faciale, polymorphic light eruption, vitiligo, seborrheic dermatitis, and fungal dermatitis.
TREATMENT Important diagnostic laboratory studies may include lupus band test (LBT), fluorescent antinuclear antibody test (ANA), anti-native DNA (n DNA), and anti-double stranded DNA (ds DNA). ANA is very sensitive, but not specific for SLE. The most specific autoantibody for SLE is n DNA or ds DNA antibody. Antibody titers tend to increase with flare-ups. Elevated sedimentation rates, elevated IgG levels and abnormal complement level abnormalities can be seen, however they may be normal in purely cutaneous forms of the disease. Patients with cutaneous lupus are often treated to prevent late pigmentary changes and scarring, as well as the rare sequelae of skin cancers arising from these lesions. Early skin lesions can often be treated effectively with oral, topical, or intralesional corticosteroids. Systemic antimalarial agents (chloro-quine and hydroxychloroquine) are helpful adjuvants in treating all the cutaneous manifestations of lupus. Surgical correction of eyelid malpositions may be necessary, but should be delayed until after quiescence of the disease.
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