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Gelerstein 2011

Topic 12. Lupus erythematosus and its variants Lupus erythematosus are a group of diseases ranging from the purely cutaneous type (discoid LE), through patterns associated with some internal problems (disseminated discoid LE and subacute cutaneous LE), to a severe multisystem disease (Systemic Lupus Erythematosus) Systemic lupus erythematosus Cause Unknown, but hereditary factors e.g. complement deficiency and certain HLA types, susceptibility. Patients with LE have autoantibodies to DNA, nuclear proteins and to other normal antigens Exposure to sunlight and artificial ultraviolet radiation (UVR), pregnancy and infection may precipitate the disease or lead to flare-ups. Some drugs, such as hydralazine and procainamide trigger SLE in a dose-dependent Presentation Typically, but not always, the onset is acute. Women > Men ~ 8:1 Classical butterfly rash with facial swelling Occasionally, a few blisters may be seen Widespread discoid papulo-squamous plaques may be seen (20% of patients, have no skin disease) Periungual telangiectasia, Erythema over the digits, hair fall, and photosensitivity Ulcers may occur on the palate, tongue or buccal mucosa Criteria for the diagnosis of SLE (must have at least 4) Malar rash Discoid plaques Photosensitivity Mouth ulcers Arthritis Serositis Renal disorder Neurological disorder Hematological disorder Immunological disorder Antinuclear antibodies (ANA) Course The skin changes may be transient, continuous or recurrent Acute SLE fever, arthritis, nephritis, polyarteritis, pleurisy, pneumonitis, pericarditis, myocarditis and involvement of the CNS. Internal involvement can be fatal. Renal involvement suggests a poor prognosis.

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Complications The skin disease may cause scarring or hyperpigmentation, but the main dangers lie with damage to other organs and the side-effects of treatment, especially systemic steroids. Differential diagnosis Malar rash sunburn, polymorphic light eruption and rosacea Discoid lesions discoid LE and Subacute cutaneous LE. psoriasis or lichen planus (occasionally) Hair fall telogen effluvium Plaques on the scalp may cause scarring alopecia. SLE should be suspect when characteristic rash is combine with fever, malaise, and internal disease Investigation: Skin biopsy: Degeneration of basal cells, epidermal thinning, inflammation around appendages Skin Immunofluorescence: Fibrillar or granular deposits of IgG, IgM, IgA and/or C3 alone in basement membrane zone Haematology: Anaemia, raised ESR, thrombocytopenia, decreased white cell count Immunology: Antinuclear antibody, antibodies to double-stranded DNA, false positive tests for syphilis, low total complement level, lupus anticoagulant factor Urine analysis: Proteinuria or haematuria, often with casts if kidneys involved Treatment Systemic steroids (prednisolone) + bed rest needed during exacerbations. Immunosuppressive agents, such as Azathioprine, cyclophosphamide and other drugs (e.g. antihypertensive therapy or anticonvulsants) may also be needed. Photosensitivity Antimalarial drugs Intermittent intravenous infusions of gamma globulin may help

Subacute cutaneous lupus erythematosus (SCLE) This is less severe than acute SLE, but is also often associated with systemic disease. Its cause is unknown, but probably involves an antibody-dependent cellular cytotoxic attack on basal cells by K cells bridged by antibody to Ro (SS-A) antigen. Presentation Photosensitivity Skin lesions are sharply marginated. Scaling psoriasiform plaques, sometimes annular, lying on the forehead, nose, cheeks, chest, hands and extensor surfaces of the arms.

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Symmetrical and are hard to tell from discoid LE, or SLE with widespread discoid lesions. Less frequent: erythrodermic, pityriasiform, exanthema like, multiforme like, TEN-like, Pityriasis rosea-like, poikilodermatosus Skin findings 1. Persisting diffuse erythema on the face 2. Morbilliform, scarlatiniform, livedo-like exanthems on the trunk 3. Patchy or diffuse erythaema on the palms and soles 4. Teleangiectatic blood vessels at the fingertips nail folds

Course Course is prolonged. The skin lesions are slow to clear but, in contrast to discoid LE, do so with little or no scarring. Complications Systemic disease is frequent, but not usually serious. Children born to mothers who have, or have had, this condition are liable to neonatal LE with transient annular skin lesions and permanent heart block. Differential diagnosis Psoriasis or widespread discoid LE. Annular lesions may resemble tinea corporis or figurate erythemas Investigations Evaluated in the same way as those with acute SLE Deposits of immunoglobulins in the skin and antinuclear antibodies in serum are present less often. Many have antibodies to the cytoplasmic antigen Ro (SS-A). Treatment Antimalarial drugs (hydroxychloroquine) Oral retinoids Systemic steroids

Discoid lupus erythematosus This is the most common form of LE. Patients with discoid LE may have one or two plaques only, or many in several areas. The cause is also unknown but UVR is one factor.

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Presentation Plaques show erythema, scaling, follicular plugging Scarring and atrophy Telangiectasia Hypopigmentation and a peripheral zone of hyperpigmentation They are well demarcated and lie mostly on sun-exposed skin of the scalp, face and ears In one variant (chilblain LE) dusky lesions appear on the fingers and toes. Course The disease may spread aggressively 50% remission over the course of several years. Scarring, Hair loss (permanently if there is scarring in scalp) Whiteness remains after the inflammation has cleared, and hypopigmentation is common in dark skinned people. Discoid LE rarely progresses to SLE. Differential diagnosis - psoriasis Discoid Lupus has larger thicker scales than Psoriasis It is more common on face, ears, and sun-exposed areas (Psoriasis - elbow, knees, scalp and sacrum.) Discoid LE is far more prone than psoriasis to scar and cause hair loss. Investigation: Most patients with discoid LE remain well. Screening for SLE and internal disease Skin biopsy is most helpful if taken from an untreated plaque where appendages are still present Direct immunofluorescence deposits of IgG, IgM, IgA and C3 at the basement membrane zone. Blood tests are usually normal but occasionally serum contains antinuclear antibodies Treatment Topical corticosteroids Topical steroids should be applied 2x a day until the lesions disappear or side-effects, such as atrophy No effect intralesional injections of triamcinolone may help. oral Antimalarial (hydroxychloroquine) Sun avoidance and sun screens protection Oral retinoids and thalidomide have proved helpful in stubborn Antinuclear antibodies ++++ + +/Sun sensitivity +++ ++++ + Internal organ involvement ++ + -

Systemic LE Subacute LE Discoid LE