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:

• Cardiac manifestations (Cardiac arrhythmia, dilated cardiomyopathy)
• Contracture of joints
• Dysphagia
• Gastrointestinal ulcers and infections
• Arthralgia
• Fever
• Malaise
• Raynaud phenomenon
• Unintentional weight loss
• Pulmonary manifestations (Interstitial lung disease)

• Weakness

  Call your provider if you have muscle weakness or other symptoms of this condition. [nlm.nih.gov]

  In this case, we report a 44-year-old man presenting with bilateral upper extremity predominant swelling and weakness. [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]

  It is said that there is an increased association with neoplasia.The Problem of DiagnosisThere are at present no generally accepted criteria for the diagnosis of polymyositis-dermatomyositis akin to the Jones criteria for rheumatic fever or to the American [doi.org]

  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

  Discussed here is the case of a 69-year-old man with a new diagnosis of dermatomyositis who was referred to a tertiary institution with an 8-month history of rash, proximal muscle weakness, fatigue and weight loss. [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]

  Adults with dermatomyositis may experience weight loss, a low-grade fever, inflamed lungs, and be sensitive to light such that the rash or muscle disease gets worse. [web.archive.org]

  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]

  Discussed here is the case of a 69-year-old man with a new diagnosis of dermatomyositis who was referred to a tertiary institution with an 8-month history of rash, proximal muscle weakness, fatigue and weight loss. [ncbi.nlm.nih.gov]

  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 Weight [childrensnational.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]

• Hypophonia

  Her symptoms progressed, including severe dysphagia, hypophonia and weakness preventing sitting. She was lost to follow-up in the public system due to financial and cultural barriers. [ncbi.nlm.nih.gov]

• Dysphagia

  Although dysphagia is a known complication of dermatomyositis, sudden onset of dysphagia without the notable aggravation of other symptoms can make the diagnosis and treatment challenging. [ncbi.nlm.nih.gov]

  Dysphagia and myositis Dysphagia is difficulty swallowing and it can be a complication of myositis. In order to swallow the body goes through three phases: oral, pharyngeal and esophageal. [belmarrahealth.com]

• Nausea

  A woman who was clinically diagnosed with dermatomyositis complained of severe pain in the right flank of the low back and abdomen, accompanied by nausea and vomiting during corticosteroid therapy. [ncbi.nlm.nih.gov]

  She had experienced nausea since starting azathioprine, this was withdrawn and her nausea settled. Three months later she was readmitted with a recurrence of her rash and myositis (CK, 666). [ard.bmj.com]

  Lymphoma, nausea, vomiting, hepatotoxicity, leukopenia, oral ulcers, thrombocytopenia Screen patients for thiopurine methyltransferase deficiency before therapy (0.3 to 11 percent of white population). [aafp.org]

  Can damage kidneys; can cause headaches, tremors and sleep difficulties; diarrhea, nausea and vomiting; high blood pressure, high blood sugar and high blood levels of potassium; increases risk of infection and lymphomas. [mda.org]

• Periorbital Edema

  edema, by violet-colored erythema of the eyelids and region over the upper eyelids, and sometimes by cutaneous vasculitis or subcutaneous calcification The specific trigger for dermatomyositis is not known, but it is thought that several cancers can [merriam-webster.com]

  edema Very frequent (present in 80%-99% of cases) Proximal muscle weakness Very frequent (present in 80%-99% of cases) Abnormal hair quantity Frequent (present in 30%-79% of cases) Abnormality of the nail Frequent (present in 30%-79% of cases) Acrocyanosis [web.archive.org]

  (Top) Heliotrope: a violaceous eruption with periorbital edema. (Bottom) Periungual telangiectasias. FIGURE 2. Characteristic findings of dermatomyositis. (Top) Heliotrope: a violaceous eruption with periorbital edema. [aafp.org]

• Papule

  In the physical examination, a reddish rash on the face and Gottron's papules on the knuckles were detected. [ncbi.nlm.nih.gov]

  These are known as Gottron's papules. [en.wikipedia.org]

  […] on extensor surfaces, particularly the elbows and joints of the hand (Gottron papules). [cmaj.ca]

• Erythema

  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]

• Eruptions

  RESULTS: The most common toxicities included cutaneous eruption, gastrointestinal upset, mucocutaneous dyspigmentation, neurologic toxicity, and retinopathy. [ncbi.nlm.nih.gov]

  Skin eruption mainly over light exposed areas Due to the above mentioned microinfarction and necrosis of the skin, the photo exposed area may get easy skin eruption. [medicaljoyworks.com]

  Gottron’s papules Heliotrope periorbital eruption: erythematous/violaceus, often edematous periorbital eruption (Figure 3) Figure 3. [clinicaladvisor.com]

  Gottron's papules Heliotrope periorbital eruption: erythematous/violaceus, often edematous periorbital eruption ( Figure 3 ) Figure 3. [dermatologyadvisor.com]

• Eczema

  (This may be mistaken for eczema .) [my.clevelandclinic.org]

  Often, the characteristic skin rashes are first misdiagnosed as eczema or psoriasis. Muscle pain can be mistaken for growing pains or overuse, and weakness can be confused with laziness or sleepiness. [jointhealth.org]

  The rash is often scaly and may look similar to eczema, but the color should set it apart from other skin conditions. This rash may get worse after sun exposure and this is often the first sign of dermatomyositis. [mctlawyers.com]

  This refers to small coin-like areas of eczema scattered over the body. These round patches of eczema are dry, red and itchy and may be mistaken for ringworm (a fungal infection). [dermnetnz.org]

• Dry Skin

  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 therapeutic option for use during an exacerbation or as maintenance [actharrheumatology.com]

  The rash may be red and patchy, like dry skin; a red or purplish color on the eyelids or cheeks that may look more like allergies; or both. Children with juvenile polymyositis do not experience skin symptoms. [myositis.org]

  Scaly, rough, dry skin, which can lead to thinning hair. Swollen, red areas around the fingernails. Hard lumps under the skin caused by calcium deposits (calcinosis). Muscle weakness in the neck, hip, back, and shoulders. [uchealth.com]

  skin, which can lead to thinning hair Swollen, red areas around the fingernails Hard lumps under the skin caused by calcium deposits (calcinosis) Muscle weakness in the neck, hip, back, and shoulders Trouble swallowing and voice changes Tiredness, fever [uhhospitals.org]

• Muscle Weakness

  Call your provider if you have muscle weakness or other symptoms of this condition. [nlm.nih.gov]

  Later she developed progressive, symmetrical proximal muscle weakness. [ncbi.nlm.nih.gov]

  From Wikidata Jump to navigation Jump to search myositis that results in inflammation located in muscle or located in skin where a skin rash is often seen prior to the onset of muscle weakness dermatopolymyositis Dermatopolymyositis, unspecified Polymyositis [wikidata.org]

• Myopathy

  BACKGROUND: Dermatomyositis (DM) is inflammatory myopathy or myositis characterized by muscle weakness and skin manifestations. In the differential diagnosis of DM the evaluation of the muscle biopsy is of importance among other parameters. [ncbi.nlm.nih.gov]

  Apolipoprotein E and apolipotroein E messenger RNA in muscle of inclusion body myositis and myopathies. Annal Neurol. 1996;40:864-72. Dalakas MC. [web.archive.org]

  […] with lung involvement Juvenile dermatopolymyositis with myopathy Clinical Information A subacute or chronic inflammatory disease of muscle and skin, marked by proximal muscle weakness and a characteristic skin rash. [icd9data.com]

• Proximal Muscle Weakness

  Later she developed progressive, symmetrical 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]

  Myalgia means muscle pain or aching. An example of a myalgia would be the ailment fibromyalgia. [belmarrahealth.com]

• Arthralgia

  Arthralgias occurred in 2 patients and Raynaud phenomenon in 4. [jamanetwork.com]

  General symptoms such as low grade fever, loss of appetite, loss of weight, arthralgia and lethargy can occur Due to the general ill health and multi-systemic involvement generalized symptoms could occur. [medicaljoyworks.com]

  […] 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]

  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]

  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]

• 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]

  She has since continued to manifest a heliotrope malar rash, erythema over her anterior superior chest and upper back, involvement of the extensor surface of the arms, and Gottron papules over the extensor surfaces of her hands, despite therapy with various [jamanetwork.com]

  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]

  Lupus erythematosus often produces what is often called a butterfly rash or malar rash, seen here on a young girl's face. This is the characteristic appearance of the butterfly rash. [mountsinai.org]

• Facial Edema

  edema, malignancies, erythrodermia, lichen planus, cutaneous vasculitis and panniculitis; rare manifestations are follicular hyperkeratosis, papular mucinosis, hypertrichosis, malignant erythema, urticaria, urticarial vasculitis, partial lipodystrophy [scielo.br]

• 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]

  […] disease secondary to autosomal dominant polycystic kidney disease, submitted to a cadaveric kidney transplant in 1992 and with normal allograft function, referred to the Nephrology Department with complaints beginning one month before consisting of asthenia [revistanefrologia.com]

  Case Report A 30-year old female patient with no relevant medical history was admitted to the Clinical Medicine Department for asthenia, debility and myalgias of approximately 45 days duration. [scielo.br]

  Clinical and functional respiratory manifestations Most patients presented with asthenia, fever, and/or weight loss (mean weight loss 10.1 5.6 kg), and dyspnoea. [erj.ersjournals.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]

• Nasal Speech

  Other signs of pharyngo-esophageal involvement include nasal speech, hoarseness, nasal regurgitation, and aspiration pneumonia. [e-ijd.org]

• Paresis

  Three weeks after the admission, the patient developed right facial central paresis, right arm hyperreflexia, and disartria. MRI ( Fig. 1 ) revealed the presence of a left frontal mass that was removed. [reumatologiaclinica.org]

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 edema 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]

• Antinuclear Autoantibodies

  Auto-antibodies A variety of auto-antibodies are associated with the disease and include Antinuclear Autoantibodies (ANA), Rheumatoid Factor (RF), and more specific myositis-associated antibodies (e.g. anti-Jo-1, anti-Mi-2, anti-SRP). [pathwaymedicine.org]

  Antinuclear autoantibodies were positive along with anti-MDA5 in association with anti-Ro52. Presence of malignancies was excluded. The diagnosis of scarcely myopathic but highly aggressive dermatomyositis was thus formulated. [clinicalmolecularallergy.biomedcentral.com]

• 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]

  Diagnostic Criteria Bohan and Peter Criteria: Symmetric proximal muscle weakness  most common symptom typical rash elevated serum muscle enzymes myopathic changes on EMG characteristic muscle biopsy abnormalities and absence of histopathologic [docsity.com]

  In total, 103 patients were excluded because of suspected s‐IBM, rhabdomyolysis or muscular dystrophy (n   73), insufficient clinical data to determine the disease course (n   18), absence of biopsy specimen (n   4) or lack of muscle biopsy abnormalities [ncbi.nlm.nih.gov]

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.

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.

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.

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. 

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. 

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

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.

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.