musculoskeletal and connective tissue disorders
Systemic Lupus Erythematosus (SLE)
Variant Forms of Lupus
DLE, also sometimes called chronic cutaneous lupus erythematosus, is a set of skin changes that can occur as part of lupus, with or without systemic involvement. Skin lesions begin as erythematous plaques and progress to atrophic scars. They cluster in light-exposed areas of the skin, such as the face, scalp, and ears. Untreated, lesions extend and develop central atrophy and scarring. There may be widespread scarring alopecia. Mucous membrane involvement may be prominent, especially in the mouth. Sometimes lesions are hypertrophic and may mimic lichen planus (called hypertrophic or verrucous lupus). This image shows discoid erythematous plaques causing atrophic scarring and resultant alopecia on the scalp. Image courtesy of Karen McKoy, MD. This photo shows chronic discoid lupus erythematosus with characteristic hyperkeratotic and erythematous plaques. © Springer Science+Business Media This image shows erythematous plaques and atrophic scars on the face resulting from discoid lupus erythematosus. Image courtesy of Karen McKoy, MD. This image shows erythematous plaques on the face resulting from discoid lupus erythematosus. Image courtesy of Karen McKoy, MD. Patients presenting with typical discoid lesions should be Diagnosis . Antibodies against dsDNA are almost invariably absent in DLE. Although it does not differentiate DLE from SLE, biopsy can rule out other disorders (eg, lymphoma or sarcoidosis). Biopsy should be done from the active margin of a skin lesion. Early treatment of DLE can prevent permanent atrophy. Exposure to sunlight or ultraviolet light should be minimized (eg, using potent sunscreens when outdoors). Topical corticosteroid ointments (particularly for dry skin) or creams (less greasy than ointments) 3 to 4 times a day (eg,triamcinoloneacetonide 0.1 or 0.5%,fluocinolone0.025 or 0.2%,flurandrenolide0.05%,betamethasonevalerate 0.1%, and, particularlybetamethasonedipropionate 0.05%) usually cause involution of small lesions; they should not be used excessively or on the face (where they cause skin atrophy). Resistant lesions can be covered with plastic tape coated withflurandrenolide. Alternatively, intradermal injection withtriamcinoloneacetonide 0.1% suspension (<0.1 mL per site) may resolve lesions, but secondary atrophy frequently follows. Antimalarials (eg, oralhydroxychloroquine5 mg/kg once a day or 2.5 mg/kg 2 times a day) are also considered first-line therapy, including for facial lesions. If first-line therapy fails, combination therapy withhydroxychloroquine200 mg/day plus oral quinacrine 50 to 100 mg once a day orhydroxychloroquineplusmethotrexate,mycophenolatemofetil, orazathioprineis used. Patients with SCLE develop extensive recurring rashes. Annular or papulosquamous lesions (psoriasiform lesions) may develop on the face, arms, and trunk. Lesions are usually photosensitive and can develop hypopigmentation but rarely scar. SCLE can be drug-induced, for example, triggered by antihypertensives (eg, diuretics, calcium channel blockers, beta blockers), proton pump inhibitors (eg,omeprazole,pantoprazole), anti-tumor necrosis factor (TNF) biologics (eg,adalimumab), and antifungal drugs (eg,terbinafine). About 30 to 62% of patients with SCLE fulfill the American College of Rheumatology's classification criteria for SLE. Arthritis and fatigue are common in SCLE, but neurologic and renal manifestations are not. Patients may be antinuclear antibody (ANA)-positive or ANA-negative. Patients usually have antibodies to Ro (SSA). Infants whose mother has Ro antibodies may have congenital SCLE or congenital heart block. Treatment . 