Lupus erythematosus (LE) refers to a diverse array of illnesses in which the immune system becomes active against an individual’s own cells, and is frequently associated with a variety of autoantibodies, including a positive ANA (antinuclear antibody). It may be isolated to the skin, involve internal organs, or both. In systemic lupus erythematosus (SLE), various internal organs may be involved, including the kidneys, central nervous system, blood cells, skin and joints. While patients with SLE frequently have skin involvement, many patients with skin involvement do not in fact have SLE or autoantibody positivity, and the diagnosis is primarily made by dermatologic exam and biopsy. Cutaneous lupus erythematosus (CLE) is typically quite photosensitive (sensitive to the sun), and there are 3 main subsets: (1) acute cutaneous lupus, otherwise known as “the butterfly rash” or “malar rash”, seen in patients who typically have obvious SLE; (2) subacute cutaneous lupus, an often widespread, red rash that is significantly photosensitive, and has a predilection for truncal and sun-exposed skin and is often associated with milder SLE if it is present at all; and (3) discoid lupus erythematosus, which is comprised of red lesions which, occur most commonly on the head and neck, and may eventuate in residual scarring and hyperpigmentation, and is most commonly present without SLE. Treatment is aimed at protection from UV light, stopping implicated medications, and control of inflammation with various topical and oral medications, including antimalarial medications (e.g. hydroxychloroquine), retinoids, and mycophenolate mofetil.

For more patient information on cutaneous lupus, see Lupus Information for Patients

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Dermatomyositis (DM) is a systemic autoimmune disease that classically causes inflammation of the skin and muscles, resulting in a skin rash and muscle weakness. However, there is a wide spectrum of organ involvement, which can range from no apparent disease to severe inflammation for each of the major systems involved—that is, skin, muscle and lungs. Dermatomyositis also may be seen in association with an underlying malignancy. Recently, several novel DM-specific autoantibodies have been described, and there is mounting evidence that these antibodies might help to further classify DM patients with regards to their clinical presentation, prognosis regarding organ involvement as well as cancer incidence, and, even response to given therapies. Treatment entails protection from UV light, identifying underlying malignancy or internal involvement if present, and control of inflammation with topical and oral medications. Systemic options for treatment include prednisone, methotrexate, mycophenolate mofetil, and IVIg. Future work is aimed at formally evaluating the efficacy and safety of specific therapies for skin, muscle, and/or lung inflammation, as well as understanding how autoantibodies may impact both classification and approach to treatment.

For more patient information on cutaneous lupus, see Dermatomyositis Information for Patients

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Systemic sclerosis

Systemic sclerosis (SSc, scleroderma) is an autoimmune connective tissue disease of unknown etiology characterized by internal organ involvement, autoantibodies and cutaneous fibrosis. It occurs either as a systemic form (systemic sclerosis) or as a localized form (morphea – see below). The systemic form has two major subsets: limited and diffuse. It often presents with Raynaud’s phenomenon (reversible vasospasm characterized by white, blue, red color changes of the digits). Severe cases may have digital ulcers. Virtually all patients have swelling and hardness of the fingers and hands (sclerodactyly). Internal organ involvement includes: GI tract, kidney, pulmonary and cardiac. The lung involvement is important because it is the most common cause of death. Interstitial lung disease (ILD) and/or pulmonary artery hypertension (PAH) need to be screened for with PFTs, HRCT and echocardiograms. Treatments with immunosuppressive drugs can be beneficial for ILD and vasoactive drugs for PAH. Scleroderma renal crisis (SRC) can also occur and can be treated with ACE inhibitors. Autoantibodies are common in SSc patients: positive ANAs with speckled or nucleolar patterns are common. Specific autoantibodies include anti-centromere autoantibodies (more common in the limited subset) and anti-topoisomerase I (Scl-70) and RNA polymerases (more common in the diffuse subset). Ultimately, the disease leads to significant cutaneous fibrosis. Options for managing cutaneous fibrosis include: protection from the cold, topical corticosteroids, UV light, vasodilators, and systemic immunosuppression with drugs such as methotrexate, mycophenolate mofetil and cyclophosphamide. None of these therapies is especially effective. Care should be taken to avoid high-dose systemic steroids, because they can cause a flare of SRC. So far the biologics have not proven very successful in SSc patients and studies are currently underway looking at newer agents.

For more patient information on cutaneous lupus, see Systemic Sclerosis Information for Patients

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Morphea is an idiopathic inflammatory skin disease, histologically similar to SSc, that affects the dermis and subcutis resulting in fibrosis and scar. It has several different subsets: plaque, linear (en coup de sabre), generalized, and profundus. It may itch or tingle, but most often is asymptomatic. Although 40% of patients may have positive ANAs, the specific autoantibodies of SSc are not seen. Morphea patients have a very low risk of ever evolving into the systemic form. Although there may be some symptoms in the area of morphea, it is not associated with internal organ involvement and has an excellent prognosis. Treatments include: topical medications, UV light, and systemic immunosuppression with methotrexate or mycophenolate mofetil.

For more patient information on cutaneous lupus, see Morphea Information for Patients

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Vasculitis refers to a group of conditions characterized by inflammation of blood vessels, and can affect the skin, kidneys, lungs, and other organs. Cutaneous vasculitis can occur in isolation or in association with other organ involvement. Vasculitis may be a primary autoimmune condition or reactive to an exogenous stimulus such a medication or an infection. The skin requires nutrients that are supplied by blood vessels of various sizes, from minute capillaries to medium-sized blood vessels. Inflammation of these structures leads to the extravasation (leakage) of blood cells and multiple clinical appearances or syndromes related to the inflammation and possibly secondary clotting. These range from minute palpable purpuric papules from capillaritis to large stellate (star-shaped) purpuric plaques, ulcers and skin necrosis from inflammation of larger vessels. Current classification and treatment of cutaneous vasculitis is based upon pathologic confirmation of vessel size, characteristics of inflammatory infiltrate, and internal organ involvement. Treatment is aimed at eliminating and/or treating potential triggers and topical and systemic therapy to control inflammation.

For more patient information on vasculitis, see Vasculitis Information for Patients

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