Sep 26, Author: This systemic disorder most frequently affects the skin and muscles but may also affect the joints; the esophagus; the lungs; and, less commonly, the heart. Cutaneous involvement may manifest as follows:.
Scaly scalp or diffuse hair loss [ 3 ]. Muscle disease may occur concurrently, may precede the skin disease, or may follow the skin disease by weeks to years. Muscle involvement manifests as the following:. Subcutaneous calcification, [ 4 ] which may result in contracture of joints; more common in children. Children may also develop a tiptoe gait secondary to flexion contracture of the ankles in early childhood, dermatomyositus and hair loss.
See Clinical Presentation for more detail. Characteristic but not pathognomonic features: Malar erythema, violaceous erythema or poikiloderma in a photosensitive distribution, violaceous erythema on the extensor surfaces, and periungual and cuticular changes.
Rare cutaneous manifestations include vesiculobullous erosive lesions and an exfoliative erythroderma, which may be more common in patients with an associated malignancy than in those without a malignancy; biopsy samples of these manifestations reveal an interface dermatitis similar to that seen in biopsy samples of heliotrope rash, Gottron papules, poikiloderma, or scalp lesions.
Distal strength, sensation, and tendon reflexes dermatomyositus and hair loss unless the patient has severely weak and atrophic muscle. Muscle enzyme levels eg, creatine kinase, aldolase, aspartate aminotransferase, lactic dehydrogenase. Imaging to screen for underlying malignancy, including CT scanning of the chest, abdomen, and pelvis, as well as transvaginal ultrasound and mammography for women.
Muscle biopsy open or via a needle: Therapy for the muscle component of dermatomyositis involves the use of corticosteroids, typically with an immunosuppressive agent. Therapy for the skin disease includes the following, among other options:. Prednisone is a first-line therapy for muscle involvement in dermatomyositis. Immunosuppressive agents eg, methotrexate, mycophenolate mofetil, azathioprine, rituximab, sirolimus.
In addition to the medications listed above, diltiazem, colchicine, alendronate, and warfarin are among the medications that have shown potential benefit in treating calcinosis.
Surgical excision of focal, tender calcinotic lesions is also considered a therapeutic option. Surgical care is usually unnecessary in the management of dermatomyositis. However, some patients may benefit from surgical removal of localized areas of calcinosis, particularly those that are painful.
See Treatment and Medication for more detail. Dermatomyositis is an idiopathic inflammatory myopathy IIM with characteristic cutaneous findings. It is a systemic disorder that most frequently affects the skin and muscles, but may also affect the joints; the esophagus; the lungs; and, less commonly, the heart.
An association between dermatomyositis and cancer has long been recognized in adult patients. InBohan and Peter first suggested a set of five criteria to aid in the diagnosis and classification of dermatomyositis. In addition to dermatomyositis, Bohan and Peter suggested the following four subsets of myositis [ 12 ]:.
In a subsequent publication, Bohan et al noted that cutaneous disease may precede the development of the myopathy in patients with dermatomyositis. Finally, another subset of patients dermatomyositus and hair loss dermatomyositis are those with controlled myopathy who continue to have severe and sometimes debilitating skin disease ie, dermatomyositus and hair loss, postmyopathic dermatomyositis.
ADM is diagnosed in patients with typical cutaneous disease dermatomyositus and hair loss show no evidence of muscle weakness and in whom serum muscle enzyme levels are repeatedly normal over a 2-year period in the absence of the use of disease-modifying black people and sun cancer such as corticosteroids, immunosuppressive agents, or both for 2 months or longer.
When studied, some ADM patients may have abnormal findings on ultrasonography, electromyography, magnetic resonance imaging MRImagnetic resonance spectroscopy, or muscle biopsy. These patients are better classified as having hypomyopathic dermatomyositis. ADM or hypomyopathic DM may also be related to an underlying malignancy. The term clinically amyopathic dermatomyositis CADM is often used to encompass patients with both amyopathic and hypomyopathic dermatomyositis.
Patients exist in whom myositis resolves after therapy but skin disease remains an active and important feature of the disorder. These patients are not classified as having ADM, even though by this point the skin lesions are the major and often only manifestation of the disease. Germani and colleagues have suggested the term postmyopathic dermatomyositis for these patients.
Therapy for the muscle component of dermatomyositis involves the use of systemic corticosteroids, with or without an immunomodulatory agent. The skin disease is treated with sun avoidance, dermatomyositus and hair loss, sunscreens, photoprotective clothing, topical corticosteroids, antimalarial agents, methotrexate, mycophenolate mofetil, or intravenous immunoglobulin IVIG.
Systemic corticosteroids are generally dermatomyositus and hair loss administered for cutaneous involvement. Rituximab may be useful in the treatment of muscle disease in dermatomyositis, and has had mixed results in treatment of skin disease, dermatomyositus and hair loss.
Physical therapy and rehabilitative measures are necessary in selected patients. Sun protective measures are necessary for patients with skin disease. Patients may visit The Myositis Association Web site for more information. Residual weakness is common, even in patients who fully recover. For discussion of dermatomyositis in pediatric patients, see Juvenile Dermatomyositis. Dermatomyositis is considered to be the result of a humoral attack against the muscle capillaries and small arterioles endothelium of the endomysial blood vessels.
Sincethere has been evidence supporting an ongoing microangiopathy. The disease starts when putative antibodies or other factors activate C3, forming C3b and C4b fragments that lead to formation of C3bNEO and membrane attack complex MACwhich are deposited in the endomysial vasculature. Complement C5b-9 MAC is deposited and is needed in preparing the cell for destruction in antibody-mediated disease.
B cells and CD4 helper cells are also present in abundance in the inflammatory reaction associated with the blood vessels, dermatomyositus and hair loss. As the disease progresses, the capillaries are destroyed, and the muscles undergo microinfarction. Perifascicular atrophy occurs in the beginning; however, as the disease advances, necrotic and degenerative fibers are present throughout the muscle.
The pathogenesis of the cutaneous component of dermatomyositis is poorly understood, but is thought to be similar to that of muscle involvement. Studies on the pathogenesis of the muscle component have been controversial.
Some suggest that the myopathy in dermatomyositis is pathogenetically different from that in polymyositis. However, other cytokine studies suggest that some of the inflammatory processes may be similar. One report has linked tumor necrosis factor TNF abnormalities with dermatomyositis. The cause of dermatomyositis is unknown. However, genetic, immunologic, infectious, and environmental factors have been implicated.
A genetic component may predispose to dermatomyositis. Polymorphisms of tumor necrosis factor TNF may be involved; specifically, the presence of the A allele is linked to photosensitivity in adults and calcinosis in children. Immunologic abnormalities are common in patients with dermatomyositis.
Patients frequently have circulating autoantibodies. Abnormal T-cell activity may be involved in the pathogenesis of both the skin disease and the muscle disease. In addition, family members may manifest other diseases associated with autoimmunity. Although their presence may help to define subtypes of dermatomyositis and polymyositis, their role in pathogenesis is uncertain. Infectious agents have been suggested as possible triggers of dermatomyositis, dermatomyositus and hair loss.
These include the following:. Cases of drug-induced dermatomyositis have been reported. Dermatomyositis-like skin changes have been reported with hydroxyurea in patients with chronic myelogenous leukemia or essential thrombocytosis.
Dermatomyositis may be initiated or exacerbated by silicone breast implants or collagen injections, dermatomyositus and hair loss, but the evidence for this is anecdotal and has not circuit training and weight loss verified in case-control studies.
One report detailed HLA differences among women in whom inflammatory myopathy developed after silicone implants, dermatomyositus and hair loss. The estimated incidence of dermatomyositis is 9.
The estimated incidence of AMD is 2. Dermatomyositis can occur in people of any age. Two peak ages dermatomyositus and hair loss onset exist: Dermatomyositis and polymyositis are twice as common in women as in men.
Neither condition shows any racial predilection. However, patients who survive the disease may experience residual weakness and disability. Children with severe dermatomyositis may develop contractures. Therefore, many patients require long-term therapy. Dermatomyositis may cause death because of muscle weakness or cardiopulmonary involvement. Patients with an associated cancer may die of the malignancy. The association between malignancy and dermatomyositis has long been recognized.
Ovarian cancer is clearly over-represented in patients with dermatomyositis; however, any malignancy may occur. Reported maligancies include lung, colon, prostate, dermatomyositus and hair loss, breast, pancreatic, cervical, dermatomyositus and hair loss, and hematologic malignancies. For example, dermatomyositus and hair loss carcinoma appears to be over-represented in certain Asian populations. In an approximately year retrospective study from southern China, 60 of dermatomyositis patients developed malignancies.
The risk of malignancy was highest in the first year after diagnosis of dermatomyositis, and nasopharyngeal carcinoma and ovarian carcinoma were the most common malignancies. Male gender, dysphagia and elevated erythrocyte sedimentation rate were risk factors for malignancy, dermatomyositus and hair loss, whereas the presence of interstitial lung disease appeared to reduce the risk of malignancy.
Calcinosis may also complicate dermatomyositis. It is rare in adults but is more common in children and has been linked to delay in diagnosis and to less-aggressive therapy. Population-based studies from British Columbia concluded that patients with dermatomyositis or polymyositis are at increased risk for venous thromboembolism deep venous thrombosis or pulmonary embolism [ 43 ] and myocardial infarction, dermatomyositus and hair loss 43 ] especially in the first year after diagnosis.
However, dermatomyositis was not associated with an increased risk of ischemic stroke. A study from Taiwan reported that the risk of osteoporosis in persons with dermatomyositis or polymyositis was 2.