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Dermatomyositis

DM

Dermatomyositis is an inflammatory disease, marked by muscle weakness and characteristic cutaneous findings of unknown etiology.


Presentation

The skin disease itself is often the first sign of dermatomyositis and considered to be the main manifestation in about 40 percent of affected people [7].

Common skin findings may include: 

  • Gottron's sign: Erythematous, scaly papules and plaques occurring in symmetric fashion particularly over the metacarpophalangeal joints, and interphalangeal joints 
  • Heliotrope rash: Erythematous rash, with or without edema, around the eyes

Muscular signs and symptoms may occur at any time of the disease course and in some cases before the dermatologic manifestations occur. Muscular involvement usually starts as a weakness during walking, when standing, when reaching above the head, arising from a sitting position or when performing similar actions.

Some of the other symptoms associated with dermatomyositis can include:

Weakness
  • In this case, we report a 44-year-old man presenting with bilateral upper extremity predominant swelling and weakness.[ncbi.nlm.nih.gov]
  • A 68-year-old man presented with polyarthritis, proximal muscle weakness, and erythema of the face, arms, neck, and anterior chest that resembled the V-neck sign.[ncbi.nlm.nih.gov]
  • It is important to consider drug-associated dermatomyositis in the differential diagnosis of patients presenting with skin lesions and muscle weakness after nivolumab treatment.[ncbi.nlm.nih.gov]
  • Later she developed progressive, symmetrical proximal muscle weakness.[ncbi.nlm.nih.gov]
  • Patient had no muscle weakness and his rash was characteristic of classic dermatomyositis. Skin biopsy was suggestive of dermatomyositis but muscle enzymes were normal. His serum was positive for anti-MDA5 antibody.[ncbi.nlm.nih.gov]
Fever
  • The addition of infliximab to glucocorticoids and tacrolimus improved the fever and hyperferritinemia and enabled a reduction in the dose of prednisolone without relapse of the diseases.[ncbi.nlm.nih.gov]
  • A 53-year-old Sri Lankan woman presented with intermittent fever of 5 months' duration with erythroderma. Later she developed progressive, symmetrical proximal muscle weakness.[ncbi.nlm.nih.gov]
  • We illustrate these challenges with the case of a 51-year-old Vietnamese-American man who initially presented with fever of unknown origin in the absence of overt skin and muscle manifestations.[ncbi.nlm.nih.gov]
  • The MSN group experienced lower dyspnea (48.2% versus 79% versus 71.4%, P 0.001) and fever (18.1% versus 39.5% versus 37.5%, P 0.01) frequencies compared to MDA5 and ARS groups.[ncbi.nlm.nih.gov]
  • Dermatomyositis and polymyositis are rare inflammatory diseases 1,2 Signs and symptoms of these conditions are varied and can have significant physiological effect on disease 3,4 Dermatomyositis Gradual onset of proximal muscle weakness Weight loss, low-grade fever[actharrheumatology.com]
Weight Loss
  • Here, we present the case of a 64-year-old Caucasian male with no significant medical history who was admitted with marked weight loss and a painful rash for 6 months.[ncbi.nlm.nih.gov]
  • […] rashes on connective skin (Gottron’s papules/sign) Roughened, dry skin on palms and fingers (mechanic’s hands) Polymyositis Gradual onset of proximal muscle weakness Fatigue, general discomfort, weight loss, low-grade fever Acthar is an FDA-approved[actharrheumatology.com]
  • Other symptoms you might experience are: muscle pain muscle tenderness problems swallowing lung problems hard calcium deposits underneath the skin, which is mostly seen in children fatigue unintentional weight loss fever There is a subtype of dermatomyositis[healthline.com]
Fatigue
  • Muscle weakness versus fatigue It may be easy to confuse muscle weakness with the general feeling of tiredness and weariness caused by fatigue. Both symptoms can occur as a result of dermatomyositis. Muscle weakness is a loss of strength.[dmpmandyou.com]
  • Related Reading: Myasthenia gravis, a neuromuscular disease, causes voluntary muscle weakness and fatigue Myasthenia gravis, a neuromuscular disease, causes voluntary muscle weakness and fatigue.[belmarrahealth.com]
  • No weakness, fatigue, fevers or rashes anywhere else on his body. He works as a chiropractor and is concerned about his patients seeing his hands.[stanfordmedicine25.stanford.edu]
  • The disease often begins with nonspecific, general symptoms (such as fever , fatigue and muscle weakness).[childrenshospital.org]
Malaise
  • Some of the other symptoms associated with dermatomyositis can include: Cardiac manifestations (Cardiac arrhythmia, dilated cardiomyopathy) Contracture of joints Dysphagia Gastrointestinal ulcers and infections Arthralgia Fever Malaise Raynaud phenomenon[symptoma.com]
  • Symptoms may include: Fever Rash around the eyelids and/or knuckles and finger joints; a rash may also occur on the elbows, knees, and ankles Muscle weakness Fatigue Malaise Muscle pain and tenderness Irritability Weight loss (due to difficulty swallowing[stanfordchildrens.org]
  • The most common symptoms include: Fever Rash around the eyelids, knuckles, or finger joints Rash on the elbows, knees, and ankles Muscle weakness Lack of energy (fatigue) Ill feeling (malaise) Muscle pain and soreness Irritable mood Trouble swallowing[stlouischildrens.org]
  • Some individuals with dermatomyositis may also develop symptoms such as low-grade fever, a general feeling of ill health (malaise), shortness of breath (dyspnea), pain in multiple joints (polyarthralgia), weight loss, and/or other abnormalities.[rarediseases.org]
Erythema
  • A 67-year-old woman exhibited chilblain-like erythema on the bilateral auricles, erythema with hyperkeratosis on the sides of fingers and the bilateral elbows, and erythema on the extensor/flexor sides of the finger joints and the iliac regions.[ncbi.nlm.nih.gov]
  • A 68-year-old man presented with polyarthritis, proximal muscle weakness, and erythema of the face, arms, neck, and anterior chest that resembled the V-neck sign.[ncbi.nlm.nih.gov]
  • Erythroderma is a severe and potentially life-threatening dermatological condition with diffuse erythema and scaling involving more than 90% of the skin surface area.[ncbi.nlm.nih.gov]
  • This patient was diagnosed with dermatomyositis due to the presence of proximal muscle weakness with abnormal electromyography and magnetic resonance imaging findings; skin lesions, such as heliotrope rash, shawl sign, and periungual erythema; and an[ncbi.nlm.nih.gov]
  • Prominent violaceous erythema with scale is visible on the chest, face, and arms. Deep-red erythema is especially visible on the side of the face. The scalp is red and scaling. ( Courtesy of Richard P.[accessmedicine.mhmedical.com]
Hyperpigmentation
  • Poikiloderma vasculare atrophicans refers to mottled hyperpigmentation and hypopigmentation of the skin with in-between telangiectases and areas of atrophy and is considered a variant of mycosis fungoides.[ncbi.nlm.nih.gov]
  • Poikiloderma, a discoloration of the skin resulting from the combination of dilated blood vessels, hyperpigmentation, hypopigmentation, and mild skin thinning. Photosensitivity, sensitivity to the sun.[hss.edu]
  • Although the skin rashes frequently fade completely, they may be followed by brownish discolorations (hyperpigmentation) of the skin, atrophy, scarring, and/or loss of color (depigmentation) of patches of skin (vitiligo).[rarediseases.org]
  • In 1956, Brunsting et al reviewed the records of 270 patients with DM at the Mayo Clinic and reported 2 cases that had generalized erythematous eruptions simulating PRP consisting of areas of hyperkeratosis and hyperpigmentation before the onset of the[the-dermatologist.com]
Muscle Weakness
  • A 68-year-old man presented with polyarthritis, proximal muscle weakness, and erythema of the face, arms, neck, and anterior chest that resembled the V-neck sign.[ncbi.nlm.nih.gov]
  • It is important to consider drug-associated dermatomyositis in the differential diagnosis of patients presenting with skin lesions and muscle weakness after nivolumab treatment.[ncbi.nlm.nih.gov]
  • Later she developed progressive, symmetrical proximal muscle weakness.[ncbi.nlm.nih.gov]
  • Patient had no muscle weakness and his rash was characteristic of classic dermatomyositis. Skin biopsy was suggestive of dermatomyositis but muscle enzymes were normal. His serum was positive for anti-MDA5 antibody.[ncbi.nlm.nih.gov]
  • weakness, classic DM (CDM) and elevated creatine kinase (CK).[ncbi.nlm.nih.gov]
Myopathy
  • The level of similarity between juvenile and adult myopathies regarding the presence of myositis-specific autoantibodies has not been fully elucidated.[ncbi.nlm.nih.gov]
  • , 6 with inclusion body myositis, 5 with immune-mediated necrotizing myopathy), 8 with muscular dystrophy (4 with dysferlinopathy, 4 with fascioscapulohumeral muscle dystrophy) and 10 healthy controls.[ncbi.nlm.nih.gov]
  • There are scarce studies in the literature about hyaluronic acid in systemic autoimmune myopathies. To analyze the serum level of hyaluronic acid in patients with dermatomyositis and polymyositis.[ncbi.nlm.nih.gov]
  • Poikilodermatomyositis is the term given to the condition with poikiloderma and inflammatory myopathy. Only a few cases are reported on erythroderma in dermatomyositis and poikilodermatomyositis.[ncbi.nlm.nih.gov]
  • Dermatomyositis is an idiopathic inflammatory myopathy known to occur as a paraneoplastic syndrome. The course of dermatomyositis is commonly reported to mirror the course of the malignancy.[ncbi.nlm.nih.gov]
Proximal Muscle Weakness
  • Later she developed progressive, symmetrical proximal muscle weakness.[ncbi.nlm.nih.gov]
  • The proximal muscle weakness, dysphagia and presence of Gottron's papules as well poikiloderma like skin changes led to the clinical diagnosis of dermatomyositis.[ncbi.nlm.nih.gov]
  • The patient presented with proximal muscle weakness, rash, elevated muscle enzyme, myopathic electromyograph and typical muscle biopsy.[ncbi.nlm.nih.gov]
  • A 68-year-old man presented with polyarthritis, proximal muscle weakness, and erythema of the face, arms, neck, and anterior chest that resembled the V-neck sign.[ncbi.nlm.nih.gov]
  • He was referred to our rheumatology outpatient clinic for rash and papules on the knuckles, face, and neck, as well as proximal muscle weakness.[ncbi.nlm.nih.gov]
Myalgia
  • Within a month after the operation, myalgia and rash, including Gottron's papules, developed, and skeletal-muscle enzymes elevated, so she was diagnosed with probable DM according to the Bohan and Peter criteria.[ncbi.nlm.nih.gov]
  • We diagnosed DM based on the presence of myalgia, Gottron's papules, and anti-aminoacyl-tRNA synthetase antibody positivity in the patient.[ncbi.nlm.nih.gov]
  • A 62-year-old woman with a 6-month history of chronic polyarthritis and myalgia presented skin lesions characteristic of DM (erythematous lesion on extensor surface of elbows, Gottron's papules, V-neck sign) with no clinical muscle impairment (global[ncbi.nlm.nih.gov]
  • Myalgia means muscle pain or aching. An example of a myalgia would be the ailment fibromyalgia .[belmarrahealth.com]
  • Dysphagia, myalgia, fever and weight loss are other features 7 . There is cell-mediated injury targeted at striated muscle with resultant atrophy, oedema, coagulation necrosis, fibrosis and calcification. Markers elevated muscle enzymes (e.g.[radiopaedia.org]
Arthralgia
  • Anti-MDA5 antibody also increased the risk of developing eight characteristics comprising Gottron's sign or papules, mechanic's hand, V rash, skin ulcers, panniculitis, alopecia, arthritis/arthralgia and pneumomediastinum, but reduced the risk of muscle[ncbi.nlm.nih.gov]
  • […] muscle-related changes on EMG: Short, small, low-amplitude polyphasic motor unit potentials Fibrillation potentials, even at rest Bizarre high-frequency repetitive discharges Positive for any of the myositis-specific autoantibodies Nondestructive arthritis or arthralgias[myositis.org]
  • Some of the other symptoms associated with dermatomyositis can include: Cardiac manifestations (Cardiac arrhythmia, dilated cardiomyopathy) Contracture of joints Dysphagia Gastrointestinal ulcers and infections Arthralgia Fever Malaise Raynaud phenomenon[symptoma.com]
  • Commonly reported signs also include myalgia and arthralgia.[orpha.net]
  • Patients most commonly present with lethargy, pruritus, fatigue, photosensitivity or arthralgias.[physio-pedia.com]
Facial Swelling
  • As the condition improves, the dose will be lowered to help reduce the side effects of steroids, which include weight gain, increased risk of infection and facial swelling.[mctlawyers.com]
  • Common side effects include weight gain, increased appetite, mood changes, increased risk for infections and facial swelling.[cincinnatichildrens.org]
  • swelling Malignancy Erythroderma Lichen planus Cutaneous vasculitis Panniculitis Rare manifestations Follicular hyperkeratosis Papular mucinosis Hypertrichosis Malignant erythema Urticaria/urticarial vasculitis Partial lipodystrophy Malignant atrophic[aafp.org]
  • swelling Gottron papules—violaceous erythematous papules overlying the dorsal interphalangeal and metacarpophalangeal joints, elbow, or patella Heliotrope discoloration of the eyelids Macular erythema of the face and forehead Mechanics’ hands—roughening[academic.oup.com]
Malar Rash
  • rash 14 (51.9%) Gottron’s sign 23 (85.2%) V Sign 16 (59.3%) Shawl Sign 15 (55.6%) Nail-bed erythema 17 (63.0%) Dilated capillary loops 14 (51.9%) Holster sign 7 (25.9%) Mechanic’s hand 6 (22.2%) Calcinosis 5 (18.5%) Raynaud’s 3 (11.1%) Myositis-specific[acrabstracts.org]
  • Skin rashes The rashes seen in JDM can affect the face with swelling around the eyes (periorbital oedema) and a purple-pink discolouration of the eyelids (heliotrope rash); there can also be redness over the cheeks (malar rash) as well as on other parts[printo.it]
Asthenia
  • Patients with malignancy, cardiac problems, asthenia-anorexia or pulmonary interstitial fibrosis are also at risk for this poor prognosis. In general, the causes of dermatomyositis are unknown.[symptoma.com]
  • Prognosis Poor prognostic indicators include recalcitrant disease, delay in diagnosis, older age, malignancy, fever, asthenia-anorexia, pulmonary interstitial fibrosis, dysphagia and leukocytosis.[aafp.org]
  • She complained from worsening arthralgias (hands, wrists, feet) and generalized asthenia but without frank muscular pain or weakness. Electromyography showed mild myopathic signs.[clinicalmolecularallergy.biomedcentral.com]

Workup

In order to diagnose dermatomyositis a person will have to go through a series of laboratory tests, electromyography, and imaging (computed tomography, ultrasonography, and chest radiography) [8]. Biopsies of the skin as well as the muscles are useful in order to confirm a diagnosis.

In older patients, there will be an assessment for malignancy once the diagnosis is confirmed. This assessment will have to be repeated on an annual basis for three years after diagnosis. In general, the testing that will be done is dependent on the patient’s age, gender, and race.

Muscle Calcification
  • ; calcific areas may be low signal; perimuscular oedema may additionally appear as high signal; signal intensity may return to normal after treatment 4 General imaging differential considerations include: polymyositis : does not affect the skin MRI T2[radiopaedia.org]
  • calcification Ocular complications including iritis, nystagmus, cotton-wool spots, optic atrophy, conjunctival edema and pseudopolyposis Internal malignancy Medical Management (current best evidence) [3] [5] The main goals when treating patients with[physio-pedia.com]
  • calcification Ocular complications including iritis, nystagmus, cotton-wool spots, optic atrophy, conjunctival edema and pseudopolyposis Internal malignancy TABLE 5 Systemic Manifestations and Complications of Dermatomyositis Systemic manifestations[aafp.org]
Creatine Phosphokinase Increased
  • Elevated creatine phosphokinase increases the suspicion of an inflammatory muscle disease, and muscle biopsy is the definitive diagnostic tool for inflammatory myopathies.[rc.rcjournal.com]
Muscle Biopsy Abnormal
  • A diagnosis of classic or definite polymyositis or dermatomyositis (Critieria A and B plus at least one of the three other criteria): Symmetrical proximal muscle weakness; Muscle biopsy abnormalities at some time during their disease: i. degeneration[clinicaltrials.gov]
  • Muscle biopsy abnormalities at some time during their disease: Muscle fiber destruction; Muscle fiber regeneration; Perivascular and interstitial inflammatory infiltrates with muscle fiber destruction. iii.[clinicaltrials.gov]

Treatment

Treatment for dermatomyositis involves treatment for the muscular manifestations and the skin disease and can range from general treatments to specific treatments that will be based on each individual case [9]. There may also be associated disorders and each subsequent problem will also need individual treatments that can vary from one patient to another.

In terms of the muscle disease, corticosteroids are usually the first line of treatment. They may be used alongside an immunosuppressive agent. For the skin disease, a topical corticosteroid and an antimalarial agent is prescribed along with a recommendation to avoid sun exposure. Medications like methotrexate and mycophenolate mofetil may also be prescribed to a patient.

In some instances, surgery might be necessary. This is usually the case when a patient needs to have a calcinosis removed [10]. Calcinosis is more often found in pediatric cases of dermatomyositis and can be prevented if the disorder is treated early and continuously in children. Inpatient care at a medical facility may also be necessary for those patients who have progressive dermatomyositis.

Prognosis

The general prognosis is fairly good, but there is a chance to develop functional disability [6]. When this disorder affects children, contractures may develop and the occurence of generalized calcinosis may influence the prognosis.

Spontaneous remission has been seen in about 20 percent of reported cases of dermatomyositis while five percent of reported cases showed severe progression. In those more severely progressive cases, the likelihood of death related to the disorder is increased. This progression and poor prognosis is most common in patients that are over the age of 60. Patients with malignancy, cardiac problems, asthenia-anorexia or pulmonary interstitial fibrosis are also at risk for this poor prognosis.

Etiology

In general, the causes of dermatomyositis are unknown. There are factors that are associated with the disorder that should be noted [2]. There are immunologic irregularities that are linked to the condition based on the fact that most patients show circulating autoantibodies. There are also polymorphisms of the tumor necrosis factors which is linked to calcinosis in children and photosensitivity in adults. With the condition there are specific genetic markers, too, which can lead to a predisposition for dermatomyositis. There is also thought to be a link between the condition and human leukocyte antigens (HLA) DR3, DR5, and DR7.

Epidemiology

While dermatomyositis has no preference for age, there is thought to be two peak ages for the onset of the disorder. In adults, this peak age is around 50-years-old while the peak age in children is between five and 10-years-old.

Dermatomyositis is reported more often in women than men but there is no racial predilection. If looking at the numbers of cases, it is seen that there are about 9.63 instances of dermatomyositis in a pool of one million people. 

Sex distribution
Age distribution

Pathophysiology

The condition itself is thought to be the result of a humoral attack on the capillaries and arterioles of the body [4]. Dermatomyositis starts after the C3 component is triggered. From there, C3b and C4b fragments form. This formation leads to the creation of C3bNE0 and the membrane attack complex. These various fragments are dropped into the endomysial vasculature along with C5b-9 MAC. This leads to endothelial cell damage, perivascular inflammation, capillary necrosis, ischemia, and destruction of muscle fibres.

At the beginning of the disease process, perifascicular atrophy is present. Then, as the condition progresses, the capillaries deteriorate and the muscles suffer from microinfarction. Necrotic and degenerative fibers will appear in later stages [5]. During the inflammation of the blood vessels, B cells and CD4 helper cells are also present. The pathophysiology of skin lesions seems to be similar. 

Prevention

Preventative measures for dermatomyositis remain unclear as the etiologic factors of the condition are not known.

Summary

Affecting the skin, muscles, joints, esophagus, lungs, and even the heart, dermatomyositis is an idiopathic inflammatory myopathy that results in visible skin problems [1]. When present in children or adolescent youths, the disorder can lead to calcinosis. There is also a link to an increased risk of certain types of cancer.

Patient Information

Dermatomyositis is an inflammatory condition that affects both children and adults. The condition presents itself as a distinct rash that is coupled with muscle weakness. It affects women more than men and is considered to have two peak ages when it manifests. In children, dermatomyositis peaks between the ages of five and 15 while it peaks between the ages of 40 and 60 in adults.

The condition’s origins are unknown so preventative measures are also not known. It is incurable but there are treatment options available for people suffering from the disorder. These options are to treat both the muscle issues and skin issues of the dermatomyositis.

References

Article

  1. Callen JP. Dermatomyositis. Lancet. Jan 1 2000;355(9197):53-7.
  2. Callen JP, Wortmann RL. Dermatomyositis. Clin Dermatol. Sep-Oct 2006;24(5):363-73.
  3. Kasteler JS, Callen JP. Scalp involvement in dermatomyositis. Often overlooked or misdiagnosed. JAMA. Dec 28 1994;272(24):1939-41.
  4. Na SJ, Kim SM, Sunwoo IN, Choi YC. Clinical characteristics and outcomes of juvenile and adult dermatomyositis. J Korean Med Sci. Aug 2009;24(4):715-21.
  5. Airio A, Pukkala E, Isomäki H. Elevated cancer incidence in patients with dermatomyositis: a population based study. J Rheumatol. Jul 1995;22(7):1300-3. 
  6. Bunch TW, Worthington JW, Combs JJ, et al. Azathioprine with prednisone for polymyositis. A controlled, clinical trial. Ann Intern Med 1980; 92:365.
  7. Dalakas MC, Illa I, Dambrosia JM, et al. A controlled trial of high-dose intravenous immune globulin infusions as treatment for dermatomyositis. N Engl J Med 1993; 329:1993.
  8. Miller FW, Leitman SF, Cronin ME, et al. Controlled trial of plasma exchange and leukapheresis in polymyositis and dermatomyositis. N Engl J Med 1992; 326:1380.
  9. Troyanov Y, Targoff IN, Tremblay JL, et al. Novel classification of idiopathic inflammatory myopathies based on overlap syndrome features and autoantibodies: analysis of 100 French Canadian patients. Medicine (Baltimore) 2005; 84:231.
  10. Hoogendijk JE, Amato AA, Lecky BR, et al. 119th ENMC international workshop: trial design in adult idiopathic inflammatory myopathies, with the exception of inclusion body myositis, 10-12 October 2003, Naarden, The Netherlands. Neuromuscul Disord 2004; 14:337.

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Last updated: 2018-06-22 09:17