Dermatomyositis (DM) is a long-term inflammatory

autoimmune disorder An autoimmune disease is a condition that results from an anomalous response of the adaptive immune system, wherein it mistakenly targets and attacks healthy, functioning parts of the body as if they were foreign organisms. It is estimated tha ...

which affects the

skin Skin is the layer of usually soft, flexible outer tissue covering the body of a vertebrate animal, with three main functions: protection, regulation, and sensation. Other animal coverings, such as the arthropod exoskeleton, have different ...

and the

muscles Muscle is a soft tissue, one of the four basic types of animal tissue. There are three types of muscle tissue in vertebrates: skeletal muscle, cardiac muscle, and smooth muscle. Muscle tissue gives skeletal muscles the ability to muscle contra ...

. Its symptoms are generally a

skin rash A rash is a change of the skin that affects its color, appearance, or texture. A rash may be localized in one part of the body, or affect all the skin. Rashes may cause the skin to change color, itch, become warm, bumpy, chapped, dry, cracked ...

and worsening muscle weakness over time. These may occur suddenly or develop over months. Other symptoms may include weight loss, fever, lung inflammation, or light sensitivity. Complications may include calcium deposits in muscles or skin. Dermatomyositis is an autoimmune disorder featuring both humoral and T-cell autoimmune processes. Dermatomyositis may develop as a paraneoplastic syndrome associated with several forms of malignancy. It is known to be associated with several viruses, especially

coxsackievirus Coxsackieviruses are a few related enteroviruses that belong to the ''Picornaviridae'' family of viral envelope, nonenveloped, linear, positive-sense single-stranded RNA viruses, as well as its genus ''Enterovirus'', which also includes poliovi ...

, but no definitive causal link has been found. Dermatomyositis is considered a type of inflammatory myopathy. Diagnosis is typically based on some combination of symptoms, blood tests,

electromyography Electromyography (EMG) is a technique for evaluating and recording the electrical activity produced by skeletal muscles. EMG is performed using an instrument called an electromyograph to produce a record called an electromyogram. An electromyo ...

, and muscle biopsies. Eighty percent of adults and sixty percent of children with juvenile dermatomyositis have a myositis-specific antibody (MSA). Although no cure for the condition is known, treatments generally improve symptoms. Treatments may include medication,

physical therapy Physical therapy (PT), also known as physiotherapy, is a healthcare profession, as well as the care provided by physical therapists who promote, maintain, or restore health through patient education, physical intervention, disease preventio ...

, exercise, heat therapy,

orthotics Orthotics () is a medical specialty that focuses on the design and application of orthoses, sometimes known as braces, calipers, or splints. An is "an externally applied device used to influence the structural and functional characteristics of ...

, assistive devices, and rest. Medications in the

corticosteroids Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex of vertebrates, as well as the synthetic analogues of these hormones. Two main classes of corticosteroids, glucocorticoids and mineralocorticoids, are invol ...

family are typically used with other agents such as

methotrexate Methotrexate, formerly known as amethopterin, is a chemotherapy agent and immunosuppressive drug, immune-system suppressant. It is used to treat cancer, autoimmune diseases, and ectopic pregnancy, ectopic pregnancies. Types of cancers it is u ...

azathioprine Azathioprine, sold under the brand name Imuran, among others, is an immunosuppressive medication. It is used for the treatment of rheumatoid arthritis, granulomatosis with polyangiitis, Crohn's disease, ulcerative colitis, and systemic lupus er ...

recommended if steroids are not working well.

Intravenous immunoglobulin Immunoglobulin therapy is the use of a mixture of antibodies (normal human immunoglobulin) to treat several health conditions. These conditions include primary immunodeficiency, immune thrombocytopenic purpura, chronic inflammatory demyelinat ...

may also improve outcomes. Most people improve with treatment and in some, the condition resolves completely. About one in 100,000 people receive a new diagnosis of dermatomyositis each year. The condition usually occurs in those in their 40s and 50s with women being affected more often than men. People of any age, however, may be affected. The condition was first described in the 1800s.

Signs and symptoms

The main symptoms include several kinds of skin

rash A rash is a change of the skin that affects its color, appearance, or texture. A rash may be localized in one part of the body, or affect all the skin. Rashes may cause the skin to change color, itch, become warm, bumpy, chapped, dry, cracke ...

along with

muscle weakness Muscle weakness is a lack of muscle strength. Its causes are many and can be divided into conditions that have either true or perceived muscle weakness. True muscle weakness is a primary symptom of a variety of skeletal muscle diseases, includ ...

in both upper arms or thighs. Although dermatomyositis is closely related to

polymyositis Polymyositis (PM) is a type of chronic inflammation of the muscles ( inflammatory myopathy) related to dermatomyositis and inclusion body myositis. Its name is derived . The inflammation of polymyositis is mainly found in the endomysial layer ...

and is sometimes assumed to be a complication of that disease, most patients with dermatomyositis develop skin symptoms before any muscle involvement.

Skin

One form the rashes take is called " heliotrope" (a purplish color) or lilac, but may also be

red Red is the color at the long wavelength end of the visible spectrum of light, next to orange and opposite violet. It has a dominant wavelength of approximately 625–750 nanometres. It is a primary color in the RGB color model and a seconda ...

. It can occur around the eyes along with swelling, but also occurs on the upper chest or back what is called the "shawl" (around the neck) or "V-sign" above the breasts and may also occur on the face, upper arms, thighs, or hands. Another form the rash takes is called Gottron's sign, which is red or violet, sometimes scaly, slightly raised

papules A papule is a small, well-defined bump in the skin. It may have a rounded, pointed or flat top, and may have a dip. It can appear with a stalk, be thread-like or look warty. It can be soft or firm and its surface may be rough or smooth. Some ...

that erupt on any of the finger joints (the

metacarpophalangeal joint The metacarpophalangeal joints (MCP) are situated between the metacarpal bones and the proximal phalanges of the fingers. These joints are of the condyloid kind, formed by the reception of the rounded heads of the metacarpal bones into shallow ...

s or the interphalangeal joints). Gottron's papules may also be found over other bony prominences including the elbows, knees, or feet. All these rashes are made worse by exposure to sunlight, and are often very itchy, painful, and may bleed. If a person exhibits only skin findings characteristic of DM, without weakness or abnormal muscle enzymes, then he or she may be experiencing amyopathic dermatomyositis (ADM), formerly known as "dermatomyositis sine myositis".

Muscles

People with DM experience progressively worsening muscle weakness in the

proximal Standard anatomical terms of location are used to describe unambiguously the anatomy of humans and other animals. The terms, typically derived from Latin or Greek roots, describe something in its standard anatomical position. This position prov ...

muscles (for example, the shoulders and thighs). Tasks that use these muscles: standing from sitting, lifting, and climbing stairs, can become increasingly difficult for people with dermatomyositis.

Respiratory

In some people, the condition affects the diaphragm muscle, the lungs directly (through inflammation), or both. This causes difficulty breathing, and dermatomyositis is considered to be a

restrictive lung disease Restrictive lung diseases are a category of extrapulmonary, pleural, or parenchymal respiratory diseases that restrict lung expansion, resulting in a decreased lung volume, an increased work of breathing, and inadequate ventilation and/or oxy ...

in patients with these symptoms. Respiratory symptoms occur in about 40% of people with dermatomyositis, and in these people, the symptoms may slowly progress and frequently are identified as an eventual cause of death. The main driver of

respiratory failure Respiratory failure results from inadequate gas exchange by the respiratory system, meaning that the arterial oxygen, carbon dioxide, or both cannot be kept at normal levels. A drop in the oxygen carried in the blood is known as hypoxemia; a r ...

in most of these patients is the damage to the lung interstitia, rather than diaphragm weakness.

Cardiac

In dermatomyositis, many patients have mild

myocarditis Myocarditis is inflammation of the cardiac muscle. Myocarditis can progress to inflammatory cardiomyopathy when there is associated ventricular remodeling and cardiac dysfunction due to chronic inflammation. Symptoms can include shortness of bre ...

and

cardiac conduction system The cardiac conduction system (CCS, also called the electrical conduction system of the heart) transmits the signals generated by the sinoatrial node – the heart's pacemaker, to cause the heart muscle to contract, and pump blood through the ...

abnormalities which may be detected if the patient undergoes special testing. However, these abnormalities typically have no symptoms or clinical consequences. In a study of 26 patients with dermatomyositis or polymyositis, only one patient reported chest pain. More rarely, these abnormalities can cause greater issues and lead to

arrhythmias Arrhythmias, also known as cardiac arrhythmias, are irregularities in the heartbeat, including when it is too fast or too slow. Essentially, this is anything but normal sinus rhythm. A resting heart rate that is too fast – above 100 beats ...

heart failure Heart failure (HF), also known as congestive heart failure (CHF), is a syndrome caused by an impairment in the heart's ability to Cardiac cycle, fill with and pump blood. Although symptoms vary based on which side of the heart is affected, HF ...

, or damage to heart valves. For those dermatomyositis patients who do have cardiac symptoms caused by the disease, their clinical course may be much worse than usual.

Other

Around 30% of people have swollen, painful joints, but this is generally mild. Later in the course of the disease, patients often experience difficulty swallowing, called

dysphagia Dysphagia is difficulty in swallowing. Although classified under " symptoms and signs" in ICD-10, in some contexts it is classified as a condition in its own right. It may be a sensation that suggests difficulty in the passage of solids or l ...

, which makes it hard to move food from the mouth to the stomach. The muscles of the

esophagus The esophagus (American English), oesophagus (British English), or œsophagus (Œ, archaic spelling) (American and British English spelling differences#ae and oe, see spelling difference) all ; : ((o)e)(œ)sophagi or ((o)e)(œ)sophaguses), c ...

may become weak, leading to reduced or irregular movements that prevent proper swallowing. Additionally, individuals might suffer from

gastroesophageal reflux disease Gastroesophageal reflux disease (GERD) or gastro-oesophageal reflux disease (GORD) is a chronic upper gastrointestinal disease in which stomach content persistently and regularly flows up into the esophagus, resulting in symptoms and/or ...

(GERD), where stomach acid flows back into the esophagus, causing heartburn and irritation. File:Dermatomyositis2.jpg, Gottron's papules on finger joints File: Dermatomyositis5.jpg, Gottron's papules on the elbows of a person with juvenile DM File: Dermatomyositis6.jpg, Gottron's papules File: Dermatomyositis8.jpg, Gottron's bumps on a person with juvenile DM File: Dermatomyositis7.jpg, Gottron's papules in a severe case of juvenile dermatomyositis File: Dermatomyositis9.jpg, Heliotrope with swelling around the eyes File: Dermatomyositis10.jpg, Heliotrope File: Dermatomyositis14.jpg, Facial rash File: Dermatomyositis16.jpg, Severe rash on the hands, extending up the forearm File: Dermatomyositis15.jpg, Forearm rash

Causes

The pathogenesis of dermatomyositis involves an autoimmune-mediated process characterized by immune complex deposition and

complement activation The complement system, also known as complement cascade, is a part of the humoral, innate immune system and enhances (complements) the ability of antibodies and phagocytic cells to clear microbes and damaged cells from an organism, promote infla ...

, leading to microangiopathy in both muscle and skin tissues. The specific mechanism of tissue injury is variable, depending on the specific

autoantibodies An autoantibody is an antibody (a type of protein) produced by the immune system that is directed against one or more of the individual's own proteins. Many autoimmune diseases (notably lupus erythematosus) are associated with such antibodies. Pr ...

and immune cell types involved. The microangiopathy has a non-uniform pattern: within a single muscle fiber, some capillaries may be heavily affected while others seem to be unharmed. In contrast to

, which pathologically centers on damage to the

endomysium The endomysium, meaning ''within the muscle'', is a wispy layer of areolar connective tissue that ensheaths each individual muscle fiber, or muscle cell. It also contains capillaries and nerves. It overlies the muscle fiber's cell membrane: th ...

of muscle fibers, dermatomyositis damages both the endomysium and the perimysium. This indicates that tissue damage from dermatomyositis is not limited to the capillaries and the tissues immediately surrounding them; it also damages the larger vessels of the muscle fibers and skin tissue, potentially causing wider structural damage. The complement system plays a crucial role in the tissue injury of dermatomyositis, through the formation and deposition of immune complexes. Autoantibodies target muscle and skin

antigens In immunology, an antigen (Ag) is a molecule, moiety, foreign particulate matter, or an allergen, such as pollen, that can bind to a specific antibody or T-cell receptor. The presence of antigens in the body may trigger an immune response. An ...

, leading to the activation of the classical complement pathway, ultimately forming the membrane attack complex (MAC) on the

endothelial The endothelium (: endothelia) is a single layer of squamous endothelial cells that line the interior surface of blood vessels and lymphatic vessels. The endothelium forms an interface between circulating blood or lymph in the lumen and the res ...

cells of microvasculature. MAC causes direct endothelial cell damage by perforating the

cell membrane The cell membrane (also known as the plasma membrane or cytoplasmic membrane, and historically referred to as the plasmalemma) is a biological membrane that separates and protects the interior of a cell from the outside environment (the extr ...

and ultimately

lysing Lysis ( ; from Greek 'loosening') is the breaking down of the membrane of a cell, often by viral, enzymic, or osmotic (that is, "lytic" ) mechanisms that compromise its integrity. A fluid containing the contents of lysed cells is called a ...

(destroying) the cell due to

osmotic Osmosis (, ) is the spontaneous net movement or diffusion of solvent molecules through a selectively-permeable membrane from a region of high water potential (region of lower solute concentration) to a region of low water potential (region o ...

effects. The destruction of vascular endothelium results in increased vascular permeability, furthering the pro-inflammatory immune cell infiltration of the tissue, and also results in tissue

ischemia Ischemia or ischaemia is a restriction in blood supply to any tissue, muscle group, or organ of the body, causing a shortage of oxygen that is needed for cellular metabolism (to keep tissue alive). Ischemia is generally caused by problems ...

due to the lack of blood flow. Additionally, complement fragments facilitate the recruitment and activation of inflammatory cells like

macrophages Macrophages (; abbreviated MPhi, φ, MΦ or MP) are a type of white blood cell of the innate immune system that engulf and digest pathogens, such as cancer cells, microbes, cellular debris and foreign substances, which do not have proteins that ...

and

T lymphocytes T cells (also known as T lymphocytes) are an important part of the immune system and play a central role in the adaptive immune response. T cells can be distinguished from other lymphocytes A lymphocyte is a type of white blood cell (leukoc ...

, further driving the inflammatory response and resulting damage to local tissue. However, the specific sequence of complement activation is not completely understood and may depend on the pathological subtype of the disease. As many as 40% of cases of dermatomyositis are paraneoplastic manifestations of an underlying cancer. The most commonly associated cancers are

ovarian cancer Ovarian cancer is a cancerous tumor of an ovary. It may originate from the ovary itself or more commonly from communicating nearby structures such as fallopian tubes or the inner lining of the abdomen. The ovary is made up of three different ...

breast cancer Breast cancer is a cancer that develops from breast tissue. Signs of breast cancer may include a Breast lump, lump in the breast, a change in breast shape, dimpling of the skin, Milk-rejection sign, milk rejection, fluid coming from the nipp ...

, and

lung cancer Lung cancer, also known as lung carcinoma, is a malignant tumor that begins in the lung. Lung cancer is caused by genetic damage to the DNA of cells in the airways, often caused by cigarette smoking or inhaling damaging chemicals. Damaged ...

overall, but the most frequent associations can vary depending on patient race or ethnicity. Inherited genetic factors play at least a partial role in developing the disease, and HLA subtypes HLA-DR3, HLA-DR52, and HLA-DR6 seem to create a disposition to autoimmune dermatomyositis.

Diagnosis

The diagnosis of dermatomyositis is based on five criteria, which are also used to differentially diagnose with respect to

: # Muscle weakness in both thighs or both upper arms # Using a

blood test A blood test is a medical laboratory, laboratory analysis performed on a blood sample that is usually extracted from a vein in the arm using a hypodermic needle, or via fingerprick. Multiple tests for specific blood components, such as a glucose ...

, finding higher levels of

enzymes An enzyme () is a protein that acts as a biological catalyst by accelerating chemical reactions. The molecules upon which enzymes may act are called substrates, and the enzyme converts the substrates into different molecules known as pro ...

found in

skeletal muscle Skeletal muscle (commonly referred to as muscle) is one of the three types of vertebrate muscle tissue, the others being cardiac muscle and smooth muscle. They are part of the somatic nervous system, voluntary muscular system and typically are a ...

, including

creatine kinase Creatine kinase (CK), also known as creatine phosphokinase (CPK) or phosphocreatine kinase, is an enzyme () expressed by various tissues and cell types. CK catalyses the conversion of creatine and uses adenosine triphosphate (ATP) to create phos ...

aldolase Fructose-bisphosphate aldolase (), often just aldolase, is an enzyme catalyzing a reversible reaction that splits the aldol, fructose 1,6-bisphosphate, into the triose phosphates dihydroxyacetone phosphate (DHAP) and glyceraldehyde 3-phosphat ...

, and glutamate oxaloacetate, pyruvate transaminases and

lactate dehydrogenase Lactate dehydrogenase (LDH or LD) is an enzyme found in nearly all living cells. LDH catalyzes the conversion of pyruvic acid, pyruvate to lactic acid, lactate and back, as it converts NAD+ to NADH and back. A dehydrogenase is an enzyme that t ...

# Using

(testing of electric signalling in muscles), finding all three of: erratic, repetitive, high-frequency signals; short, low-energy signals between skeletal muscles and

motor neurons A motor neuron (or motoneuron), also known as efferent neuron is a neuron whose cell body is located in the motor cortex, brainstem or the spinal cord, and whose axon (fiber) projects to the spinal cord or outside of the spinal cord to directly or ...

that have multiple phases; and sharp activity when a needle is inserted into the muscle # Examining a

muscle biopsy In medicine, a muscle biopsy is a procedure in which a piece of muscle tissue is removed from an organism and examined microscopically. A muscle biopsy can lead to the discovery of problems with the nervous system, connective tissue, vascular s ...

under a microscope and finding mononuclear white blood cells between the muscle cells, and finding abnormal muscle cell degeneration and regeneration, dying muscle cells, and muscle cells being consumed by other cells (

phagocytosis Phagocytosis () is the process by which a cell (biology), cell uses its plasma membrane to engulf a large particle (≥ 0.5 μm), giving rise to an internal compartment called the phagosome. It is one type of endocytosis. A cell that performs ph ...

) #

Rash A rash is a change of the skin that affects its color, appearance, or texture. A rash may be localized in one part of the body, or affect all the skin. Rashes may cause the skin to change color, itch, become warm, bumpy, chapped, dry, cracke ...

es typical of dermatomyositis, which include heliotrope rash, Gottron sign, and Gottron papules The fifth criterion is what differentiates dermatomyositis from polymyositis; the diagnosis is considered definite for dermatomyositis if three of items 1 through 4 are present in addition to 5, probable with any two in addition to 5, and possible if just one is present in addition to 5. Dermatomyositis is associated with

, especially antinuclear antibodies (ANA). Around 80% of people with DM test positive for ANA and around 30% of people have myositis-specific autoantibodies which include antibodies to aminoacyl-tRNA synthetases ( anti-synthetase antibodies), including antibodies against histidine—tRNA ligase ( Anti-Jo1); antibodies to

signal recognition particle The signal recognition particle (SRP) is an abundant, cytosolic, universally conserved ribonucleoprotein (protein-RNA complex) that recognizes and targets specific proteins to the endoplasmic reticulum in eukaryotes and the plasma membrane ...

(SRP); and anti-Mi-2 antibodies.

Magnetic resonance imaging Magnetic resonance imaging (MRI) is a medical imaging technique used in radiology to generate pictures of the anatomy and the physiological processes inside the body. MRI scanners use strong magnetic fields, magnetic field gradients, and ...

may be useful to guide muscle biopsy and to investigate involvement of internal organs;

X-ray An X-ray (also known in many languages as Röntgen radiation) is a form of high-energy electromagnetic radiation with a wavelength shorter than those of ultraviolet rays and longer than those of gamma rays. Roughly, X-rays have a wavelength ran ...

may be used to investigate joint involvement and

calcification Calcification is the accumulation of calcium salts in a body tissue. It normally occurs in the formation of bone, but calcium can be deposited abnormally in soft tissue,Miller, J. D. Cardiovascular calcification: Orbicular origins. ''Nature M ...

s. A given case of dermatomyositis may be classified as amyopathic dermatomyositis if only skin is affected and no muscle weakness for longer than 6 months is seen according to one 2016 review, or two years according to another.

Classification

Dermatomyositis is a form of systemic

connective tissue disorder Connective tissue diseases (also termed connective tissue disorders, or collagen vascular diseases), are medical conditions that affect connective tissue. Connective tissues protect, support, and provide structure for the body's other tissues a ...

, a class of diseases that often involves autoimmune dysfunction. It has also been classified as an idiopathic inflammatory myopathy, along with polymyositis, necrotizing autoimmune myositis, cancer-associated myositis, and sporadic inclusion body myositis. A form of this disorder that occurs prior to adulthood is known as juvenile dermatomyositis.

Treatment

No cure for dermatomyositis is known, but the symptoms can be treated. Options include medication, physical therapy, exercise, heat therapy (including microwave and ultrasound), orthotics and assistive devices, and rest. The standard treatment for dermatomyositis is a

corticosteroid Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex of vertebrates, as well as the synthetic analogues of these hormones. Two main classes of corticosteroids, glucocorticoids and mineralocorticoids, are invo ...

drug, given either in pill form or intravenously. Immunosuppressant drugs, such as

and

, may reduce inflammation in people who do not respond well to

prednisone Prednisone is a glucocorticoid medication mostly used to immunosuppressive drug, suppress the immune system and decrease inflammation in conditions such as asthma, COPD, and rheumatologic diseases. It is also used to treat high blood calcium ...

. Periodic treatment using

intravenous immunoglobulin Immunoglobulin therapy is the use of a mixture of antibodies (normal human immunoglobulin) to treat several health conditions. These conditions include primary immunodeficiency, immune thrombocytopenic purpura, chronic inflammatory demyelinat ...

can also improve recovery. Other immunosuppressive agents used to treat the inflammation associated with dermatomyositis include cyclosporine A,

cyclophosphamide Cyclophosphamide (CP), also known as cytophosphane among other names, is a medication used as chemotherapy and to suppress the immune system. As chemotherapy it is used to treat lymphoma, multiple myeloma, leukemia, ovarian cancer, breast cancer ...

, and

tacrolimus Tacrolimus, sold under the brand name Prograf among others, is an immunosuppressive drug. After Allotransplantation, allogenic organ transplant, the risk of organ Transplant rejection, rejection is moderate. To lower the risk of organ rejectio ...

. Physical therapy is usually recommended to prevent muscle atrophy and to regain muscle strength and range of motion. Many individuals with dermatomyositis may need a topical ointment, such as topical corticosteroids, for their skin disorder. They should wear high-protection sunscreen and protective clothing. Surgery may be required to remove calcium deposits that cause nerve pain and recurrent infections.

Antimalarial Antimalarial medications or simply antimalarials are a type of antiparasitic chemical agent, often naturally derived, that can be used to treat or to prevent malaria, in the latter case, most often aiming at two susceptible target groups, young ...

medications, especially

hydroxychloroquine Hydroxychloroquine, sold under the brand name Plaquenil among others, is a medication used to prevent and treat malaria in areas where malaria remains sensitive to chloroquine. Other uses include treatment of rheumatoid arthritis, lupus, an ...

and

chloroquine Chloroquine is an antiparasitic medication that treats malaria. It works by increasing the levels of heme in the blood, a substance toxic to the malarial parasite. This kills the parasite and stops the infection from spreading. Certain types ...

, are used to treat the rashes, as is done for similar conditions.

Rituximab Rituximab, sold under the brand name Rituxan among others, is a monoclonal antibody medication used to treat certain autoimmune diseases and types of cancer. It is used for non-Hodgkin lymphoma, chronic lymphocytic leukemia (in children and ad ...

is used when people do not respond to other treatments. As of 2016, treatments for amyopathic dermatomyositis in adults did not have a strong evidence base; published treatments included antimalarial medications,

steroids A steroid is an organic compound with four fused rings (designated A, B, C, and D) arranged in a specific molecular configuration. Steroids have two principal biological functions: as important components of cell membranes that alter mem ...

, taken or orally or applied to the skin,

calcineurin inhibitors Immunosuppressive drugs, also known as immunosuppressive agents, immunosuppressants and antirejection medications, are drugs that inhibit or prevent the activity of the immune system. Classification Immunosuppressive drugs can be classified ...

applied to the skin,

dapsone Dapsone, also known as 4,4'-sulfonyldianiline (SDA) or diaminodiphenyl sulfone (DDS), is an antibiotic commonly used in combination with rifampicin and clofazimine for the treatment of leprosy. It is a second-line medication for the treatment an ...

, and mycophenolate mofetil. None appears to be very effective; among them, intravenous immunoglobulin has had the best outcomes.

Prognosis

The cutaneous manifestations of dermatomyositis may or may not improve with therapy in parallel with the improvement of the myositis. In some people, the weakness and rash resolve together. In others, the two are not linked, with one or the other being more challenging to control. Often, cutaneous disease persists after adequate control of the muscle disease. The risk of death from the condition is much higher if the heart or lungs are affected. Before the advent of modern treatments such as prednisone, intravenous immunoglobulin,

plasmapheresis Plasmapheresis (from the Greek language, Greek πλάσμα, ''plasma'', something molded, and ἀφαίρεσις ''aphairesis'', taking away) is the removal, treatment, and return or exchange of blood plasma or components thereof from and to the ...

chemotherapies Chemotherapy (often abbreviated chemo, sometimes CTX and CTx) is the type of cancer treatment that uses one or more anti-cancer drugs ( chemotherapeutic agents or alkylating agents) in a standard regimen. Chemotherapy may be given with a cura ...

, and other drugs, the prognosis was poor.Page 285 in: Thomson and Cotton ''Lecture Notes in Pathology'', Blackwell Scientific. Third Edition

Epidemiology

Incidence of DM peaks at ages 40–50, but the disease can affect people of all ages. It tends to affect more women than men. The prevalence of DM ranges from one to 22 per 100,000 people.

History

The diagnostic criteria were proposed in 1975 and became widely adopted. Amyopathic DM, also called DM sine myositis, was named in 2002.

People who were affected with dermatomyositis

* Opera singer

Maria Callas Maria Callas (born Maria Anna Cecilia Sophia Kalogeropoulos; December 2, 1923 – September 16, 1977) was an American-born Greek soprano and one of the most renowned and influential opera singers of the 20th century. Many critics praised ...

(1923–1977) allegedly had dermatomyositis from 1975 until her death. * Actor

Laurence Olivier Laurence Kerr Olivier, Baron Olivier ( ; 22 May 1907 – 11 July 1989) was an English actor and director. He and his contemporaries Ralph Richardson and John Gielgud made up a trio of male actors who dominated the British stage of the m ...

(1907–1989) had dermatomyositis from 1974 until his death. *

American football American football, referred to simply as football in the United States and Canada and also known as gridiron football, is a team sport played by two teams of eleven players on a rectangular American football field, field with goalposts at e ...

running back A running back (RB) is a member of the offensive backfield in gridiron football. The primary roles of a running back are to receive American football plays#Offensive terminology, handoffs from the quarterback to Rush (American football)#Offense ...

Ricky Bell (1955–1984), the runner-up for the

Heisman Trophy The Heisman Memorial Trophy ( ; also known simply as the Heisman) is awarded annually since 1935 to the top player in college football. It is considered the most prestigious award in the sport and is presented by the Heisman Trophy Trust followin ...

in 1976, and the number-one pick in the

NFL draft The NFL draft, officially known as the Annual Player Selection Meeting, is an annual event which serves as the most common source of player recruitment in the National Football League. Each team is given a position in the drafting order in reve ...

in 1977, died at the age of 29 from heart failure caused by this disease. * Rob Buckman (1948–2011) a doctor, comedian, and author, and the president of the Humanist Association of Canada. * Samantha, Indian actress diagnosed in 2022. * Suhani Bhatnagar (2005–2024), Indian actress. * Tim Rooney (1947-2006), American actor and son of Mickey Rooney.

Research

As of 2016, research was ongoing into causes for DM, as well as

biomarkers In biomedical contexts, a biomarker, or biological marker, is a measurable indicator of some biological state or condition. Biomarkers are often measured and evaluated using blood, urine, or soft tissues to examine normal biological processes, p ...

; clinical trials were ongoing for use of the following drugs in DM: ajulemic acid (Phase II),

adrenocorticotropic hormone Adrenocorticotropic hormone (ACTH; also adrenocorticotropin, corticotropin) is a polypeptide tropic hormone produced by and secreted by the anterior pituitary gland. It is also used as a medication and diagnostic agent. ACTH is an important ...

gel (Phase IV, open label), IMO-8400, an antagonist of Toll-like receptor 7,8 and 9 (Ph II), abatacept (Phase IV, open label), and

sodium thiosulfate Sodium thiosulfate (sodium thiosulphate) is an inorganic compound with the formula . Typically it is available as the white or colorless pentahydrate (x = 5), which is a white solid that dissolves well in water. The compound is a reducing agent an ...

(Phase II).

References

External links

{{Authority control Autoimmune diseases Connective tissue diseases Disorders of fascia Steroid-responsive inflammatory conditions Systemic connective tissue disorders Wikipedia medicine articles ready to translate Wikipedia neurology articles ready to translate Idiopathic diseases