Dermatomyositis

Rheumatology

Clinicals - History

Fact Explanation
Introduction Dermatomyositis is an extremely rare connective tissue disorder and basically involves the muscles and skin. Rarely joints, oesophagus, lungs and heart also can be affected. The pathological process is mainly via idiopathic inflammation of the muscles with associated skin manifestations. Both children and adults can suffer from the illness. Patients with the illness usually presents with the skin manifestations and will have features of proximal muscle weakness with muscle involvement. Both muscle weakness and skin lesions could occur simultaneously or one may precede the other. Introduction
Dermatomyositis is an extremely rare connective tissue disorder and basically involves the muscles and skin. Rarely joints, oesophagus, lungs and heart also can be affected. The pathological process is mainly via idiopathic inflammation of the muscles with associated skin manifestations. Both children and adults can suffer from the illness. Patients with the illness usually presents with the skin manifestations and will have features of proximal muscle weakness with muscle involvement. Both muscle weakness and skin lesions could occur simultaneously or one may precede the other.
Pathophysiology The entire pathophysiology of dermatomysitis is yet to be completed. Immune mediated inflammatory myopathy is the basic to the current knowledge. Inflammation is via activation of complement pathway and formation of membrane attack complex and other inflammatory mediators which causes inflammation and destruction of microvasculature of the muscles and the skin. This leads to microinfarction, perfascicular atrophy and necrosis of muscle tissue. Similar pathology is thought to be occurred in skin as well. Pathophysiology
The entire pathophysiology of dermatomysitis is yet to be completed. Immune mediated inflammatory myopathy is the basic to the current knowledge. Inflammation is via activation of complement pathway and formation of membrane attack complex and other inflammatory mediators which causes inflammation and destruction of microvasculature of the muscles and the skin. This leads to microinfarction, perfascicular atrophy and necrosis of muscle tissue. Similar pathology is thought to be occurred in skin as well.
Features of proximal muscle weakness This may precede, follow or occur concurrently with cutaneous manifestations. The mayopathy commonly affects proximal muscle groups and patient may complain difficulties in carrying out activities involving proximal muscle groups. They may complain the difficulty in standing from seated or squatting position, they might need support. They may feel difficulties when climbing stairs and mountains. Regarding the upper limb weakness they may feel the difficulty when reaching an object in a higher place, combing hair or taking of suits and similar activities. The muscles could be tender though it is not typical. In early child hood patient may develop tip toe gait due to contracture at ankle. Features of proximal muscle weakness
This may precede, follow or occur concurrently with cutaneous manifestations. The mayopathy commonly affects proximal muscle groups and patient may complain difficulties in carrying out activities involving proximal muscle groups. They may complain the difficulty in standing from seated or squatting position, they might need support. They may feel difficulties when climbing stairs and mountains. Regarding the upper limb weakness they may feel the difficulty when reaching an object in a higher place, combing hair or taking of suits and similar activities. The muscles could be tender though it is not typical. In early child hood patient may develop tip toe gait due to contracture at ankle.
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. 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.
Skin eruption of other areas Eruption of the upper outer thigh, mid face, eye lid margins and dorsum of the hands would be typical areas. These lesions could be itchy and may be intense. Skin eruption of other areas
Eruption of the upper outer thigh, mid face, eye lid margins and dorsum of the hands would be typical areas. These lesions could be itchy and may be intense.
Changes in fingers and nail folds Some times the patient may notice nail and finger changes which are typical for dermatomyositis which are described latter. [10 Changes in fingers and nail folds
Some times the patient may notice nail and finger changes which are typical for dermatomyositis which are described latter. [10
Scaly scalp lesions and diffuse hair loss As in many connective tissue disorders this also could lead to hair loss. Scaly scalp lesions and diffuse hair loss
As in many connective tissue disorders this also could lead to hair loss.
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. 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.
Difficulty in swallowing Due to the weak skeletal muscle of the oesophagus dysphagia can occur. Difficulty in swallowing
Due to the weak skeletal muscle of the oesophagus dysphagia can occur.
Gastric reflux features Gastric and chest burn with regurgitation can occur due to sphincter malfunction due to weakness of the smooth muscles of the oesophagus. Gastric reflux features
Gastric and chest burn with regurgitation can occur due to sphincter malfunction due to weakness of the smooth muscles of the oesophagus.
Features of heart failure Due to the involvement of cardiac muscles there may be patients with cardiomyopathy. Exertional dyspnoea, leg swelling and abdominal fullness may occur. Features of heart failure
Due to the involvement of cardiac muscles there may be patients with cardiomyopathy. Exertional dyspnoea, leg swelling and abdominal fullness may occur.
Features of arrythmia Similarly due to the cardiac involvement varying types of arrythmia can occur causing palpitation, syncopal attacks. Features of arrythmia
Similarly due to the cardiac involvement varying types of arrythmia can occur causing palpitation, syncopal attacks.
Features of respiratory difficulties Due to the involvement of respiratory muscles and interstitial lung involvement breathing difficulties could occur. Features of respiratory difficulties
Due to the involvement of respiratory muscles and interstitial lung involvement breathing difficulties could occur.
Evidence of a malignancy Dermatomyositis could be a manifestation of an internal malignancy specially in adults. Therefore a thorough history should be taken in relation to evidence of concurrent or previous malignant diseases. Evidence of a malignancy
Dermatomyositis could be a manifestation of an internal malignancy specially in adults. Therefore a thorough history should be taken in relation to evidence of concurrent or previous malignant diseases.

Clinicals - Examination

Fact Explanation
Dermatological manifestation: Heliotrope Rash This is one of the pathognomonic cutaneous lesion of dermatomyositis. It involves the periorbital skin simultaneously. It is typically a violaceous to erythematous rash which could be usually associated with itching and swelling. Dermatological manifestation: Heliotrope Rash
This is one of the pathognomonic cutaneous lesion of dermatomyositis. It involves the periorbital skin simultaneously. It is typically a violaceous to erythematous rash which could be usually associated with itching and swelling.
Dermatological manifestation: Gottron papules Gottron papules are also another pathognomonic lesion in dermatomyositis involving the bony prominences specially those of hands (metacarpophalengeal and interphalengeal joints). It is a flat-topped, erythematous to violaceous papules which may resemble psoriasis or lesions in lupus erythromatosus. Dermatological manifestation: Gottron papules
Gottron papules are also another pathognomonic lesion in dermatomyositis involving the bony prominences specially those of hands (metacarpophalengeal and interphalengeal joints). It is a flat-topped, erythematous to violaceous papules which may resemble psoriasis or lesions in lupus erythromatosus.
Dermatological manifestation: Photo sensitive rashes (poikiloderma) in photo exposed areas Poikiloderma has been labeled for associated changes in pigmentation, telangiactesis and erythema of the skin which can be more obvious over photo exposed areas. According to the different areas of the lesion different names have been given as physical signs. The upper back (shawl sign), lateral thighs (holster sign) and upper chest and arm in 'V' configuration. Photoexposed areas could be eruptive and itchy even. Dermatological manifestation: Photo sensitive rashes (poikiloderma) in photo exposed areas
Poikiloderma has been labeled for associated changes in pigmentation, telangiactesis and erythema of the skin which can be more obvious over photo exposed areas. According to the different areas of the lesion different names have been given as physical signs. The upper back (shawl sign), lateral thighs (holster sign) and upper chest and arm in 'V' configuration. Photoexposed areas could be eruptive and itchy even.
Dermatological manifestation: Mechanic's hands This consists of rough, cracked skin at the tips and lateral aspects of the fingers creating irregular dirty-appearing markings that mimic those in a mechanic. This sign is also seen in polymyositis. Presence of mechanic's hand as an association with high prevalence of lung diseases as well. Dermatological manifestation: Mechanic's hands
This consists of rough, cracked skin at the tips and lateral aspects of the fingers creating irregular dirty-appearing markings that mimic those in a mechanic. This sign is also seen in polymyositis. Presence of mechanic's hand as an association with high prevalence of lung diseases as well.
Dermatological manifestation: Calcinosis Calcinosis of soft tissues could occur in paediatric patients with the disease which could be obvious at finger tips as well as other areas of the skin. Dermatological manifestation: Calcinosis
Calcinosis of soft tissues could occur in paediatric patients with the disease which could be obvious at finger tips as well as other areas of the skin.
Dermatological manifestation: Raynods phenomenon Bluish discoloration of the extremities when exposed cold is characterized as Raynods phenomenon and also can be seen in connective tissue disorders including dermatomyositis. Dermatological manifestation: Raynods phenomenon
Bluish discoloration of the extremities when exposed cold is characterized as Raynods phenomenon and also can be seen in connective tissue disorders including dermatomyositis.
Manifestation of skeletal muscle weakness Mainly the proximal muscles are affected with preserved distal muscle strength. Extensors of the upper limb are commonly affected. Proximal muscle power will be reduced and there may be difficulties in standing from seated or squatting position, combing and similar activities. There may be weak trunk muscles as well. Sensation is preserved and tendon reflexes may be normal unless severely affected. During early phase of the illness muscles would be tender. Manifestation of skeletal muscle weakness
Mainly the proximal muscles are affected with preserved distal muscle strength. Extensors of the upper limb are commonly affected. Proximal muscle power will be reduced and there may be difficulties in standing from seated or squatting position, combing and similar activities. There may be weak trunk muscles as well. Sensation is preserved and tendon reflexes may be normal unless severely affected. During early phase of the illness muscles would be tender.
Cardiac involvement with features of heart failure Due to the cardiac muscle involvement and cardiomayopathy cardiac failure may follow. Ankle edema, ascites, increased Jugular Venous Pressure, pulmonary edema and 3rd heart soud may be obvious. If arrythmias exist pulse may be irregular. Cardiac involvement with features of heart failure
Due to the cardiac muscle involvement and cardiomayopathy cardiac failure may follow. Ankle edema, ascites, increased Jugular Venous Pressure, pulmonary edema and 3rd heart soud may be obvious. If arrythmias exist pulse may be irregular.
Joint involvement Poly arthritis may occur with evidence of inflammation mainly involving the small joints of the hand. Joint involvement
Poly arthritis may occur with evidence of inflammation mainly involving the small joints of the hand.
Evidence of malignancy Thorough systemic examination should carried out to detect hidden malignancies and evidence of secondaries and lymph nodes as dermatomyositis could be a manifestation of a malignancy. Evidence of malignancy
Thorough systemic examination should carried out to detect hidden malignancies and evidence of secondaries and lymph nodes as dermatomyositis could be a manifestation of a malignancy.

Investigations - Diagnosis

Fact Explanation
Basic blood investigations and ECG Full blood count might show mild anaemia which is usually due to anaemia of chronic disease. Other inflammatory markers such as ESR and CRP will be elevated. ECG will show the associated arrhythmia and features of ventricular dysfunction. Basic blood investigations and ECG
Full blood count might show mild anaemia which is usually due to anaemia of chronic disease. Other inflammatory markers such as ESR and CRP will be elevated. ECG will show the associated arrhythmia and features of ventricular dysfunction.
Electromyography Electromyography will show characteristic findings for inflammatory myopathy tho it is non specific and not diagnostic. Electromyography
Electromyography will show characteristic findings for inflammatory myopathy tho it is non specific and not diagnostic.
Muscle enzyme levels Most of the time muscle enzyme levels would be elevated. Main muscle enzyme which is widely available is creatine kinase level which is more sensitive and more specific. Other enzyme levels usually altered are aldolase, aspartate aminotransferase and lactate dehydrogenase. Muscle enzyme levels
Most of the time muscle enzyme levels would be elevated. Main muscle enzyme which is widely available is creatine kinase level which is more sensitive and more specific. Other enzyme levels usually altered are aldolase, aspartate aminotransferase and lactate dehydrogenase.
Serological investigations Though the serology is not routinely used for diagnosis those can be performed for classification to subtypes and for prognostification. These antibodies are generally called as myositis specific antibodies (MSA).

Anti nuclear antibody (ANA) will be positive in most patients though it is not essential for the diagnosis.

Other documented MSA auto anti bodies are Anti-Mi-02 and Anti-Jo-01.
Serological investigations
Though the serology is not routinely used for diagnosis those can be performed for classification to subtypes and for prognostification. These antibodies are generally called as myositis specific antibodies (MSA).

Anti nuclear antibody (ANA) will be positive in most patients though it is not essential for the diagnosis.

Other documented MSA auto anti bodies are Anti-Mi-02 and Anti-Jo-01.
Imaging Imaging would start from simple chest x-ray joint x ray. Chest xray may show associated interstitial lung diseases, cardiomegaly with cardiac involvement, features of heart failure, features of a malignant association such as pleural effusion, malignant pulmonary shadows and lymphadenopathy. Join xray may show

Ultra sound scan of the muscle can be done to evaluate the status of muscle. Ultrasound of the neck, chest and abdomen can be performed to see undetected malignancies and lymph nodes which may be associated with the disease. Trans vaginal scan would be helpful in detecting gynaecological malignancies and ultrasound breast and mammography has to be performed if a breast cancer is suspected.

CT scan of the neck , thorax, abdomen and pelvis has to be carried out if a malignancy is highly suspected.

MRI scan of the affected muscles would be beneficial in evaluating the muscle inflammation and for differential diagnosis.
Imaging
Imaging would start from simple chest x-ray joint x ray. Chest xray may show associated interstitial lung diseases, cardiomegaly with cardiac involvement, features of heart failure, features of a malignant association such as pleural effusion, malignant pulmonary shadows and lymphadenopathy. Join xray may show

Ultra sound scan of the muscle can be done to evaluate the status of muscle. Ultrasound of the neck, chest and abdomen can be performed to see undetected malignancies and lymph nodes which may be associated with the disease. Trans vaginal scan would be helpful in detecting gynaecological malignancies and ultrasound breast and mammography has to be performed if a breast cancer is suspected.

CT scan of the neck , thorax, abdomen and pelvis has to be carried out if a malignancy is highly suspected.

MRI scan of the affected muscles would be beneficial in evaluating the muscle inflammation and for differential diagnosis.
Skin and Muscle biopsy for histological evaluation Biopsy can be performed to aid the diagnosis, for differential diagnosis and for detecting steroid induced myopathy.
Skin biopsy would show interface dermatitis which is similar to lupus erythematosus.
Muscle biopsy can be performed by a needle or as an open biopsy. Muscle biopsy is diagnostic and will show perivascular and interfascicular inflammatory infiltrates with associated degeneration of muscle fibers.
Skin and Muscle biopsy for histological evaluation
Biopsy can be performed to aid the diagnosis, for differential diagnosis and for detecting steroid induced myopathy.
Skin biopsy would show interface dermatitis which is similar to lupus erythematosus.
Muscle biopsy can be performed by a needle or as an open biopsy. Muscle biopsy is diagnostic and will show perivascular and interfascicular inflammatory infiltrates with associated degeneration of muscle fibers.

Investigations - Management

Fact Explanation
Full blood count During the management FBC will show the associated anaemia which is usually due to anaemia of chronic disease and iron deficiency anaemia. This has to corrected promptly as it can aggravate the existing cardiac failure. Increased white cell count will be elevated in case of infection which has to be treated accordingly because the managing immunosuppressants could cause infection. Full blood count
During the management FBC will show the associated anaemia which is usually due to anaemia of chronic disease and iron deficiency anaemia. This has to corrected promptly as it can aggravate the existing cardiac failure. Increased white cell count will be elevated in case of infection which has to be treated accordingly because the managing immunosuppressants could cause infection.
Inflammatory markers (ESR/CRP) These would be elevated in disease flair up and with ongoing infection. So the detection of them can be made. Inflammatory markers (ESR/CRP)
These would be elevated in disease flair up and with ongoing infection. So the detection of them can be made.
ECG ECG should be monitored during the course of treatment because it shows the status of associated arrhythmias. ECG
ECG should be monitored during the course of treatment because it shows the status of associated arrhythmias.
2D Echo cardiogram As cardiomayopathy can be associated with the illness follow up 2D Echo should be performed to see the cardiac status. 2D Echo cardiogram
As cardiomayopathy can be associated with the illness follow up 2D Echo should be performed to see the cardiac status.
Muscle enzymes During flair up of the disease the muscle enzymes specially creatine kinase level will be elevated. Muscle enzymes
During flair up of the disease the muscle enzymes specially creatine kinase level will be elevated.
Imaging and malignant markers for detecting associated malignancy Imaging and malignant markers such as CA-125, CEA, CA-19.9 should be carried out annually for 3 years in adult patients. Imaging and malignant markers for detecting associated malignancy
Imaging and malignant markers such as CA-125, CEA, CA-19.9 should be carried out annually for 3 years in adult patients.
Liver function tests,renal function tests and urine full report Serum creatine, AST, ALT and UFR has to be performed to see occurrence of renal or liver impairment which could be due to the concurrent other auto immune pathologies or due to the treatment. Liver function tests,renal function tests and urine full report
Serum creatine, AST, ALT and UFR has to be performed to see occurrence of renal or liver impairment which could be due to the concurrent other auto immune pathologies or due to the treatment.

Management - Supportive

Fact Explanation
Health education The patient should be aware about the disease including the rarity of it. Patient should know the prognosis, disease progress, avoidance factors, treatment modalities and side effects of long term treatment. Not only the patient all the family members with the care givers should participate in the discussion as their support is more important to the patient. Health education
The patient should be aware about the disease including the rarity of it. Patient should know the prognosis, disease progress, avoidance factors, treatment modalities and side effects of long term treatment. Not only the patient all the family members with the care givers should participate in the discussion as their support is more important to the patient.
Avoidance of exposure to sunlight Photo sensitivity rashes may be distressing to the patient. So advice should be given regarding the avoidance of sunlight and applying sunscreens.Photo protective clothing is useful. Avoidance of exposure to sunlight
Photo sensitivity rashes may be distressing to the patient. So advice should be given regarding the avoidance of sunlight and applying sunscreens.Photo protective clothing is useful.
Adequate rest When the muscles are severely inflamed and when the cardiac failure with cardiomyopathy is associated adequate rest should be there. Adequate rest
When the muscles are severely inflamed and when the cardiac failure with cardiomyopathy is associated adequate rest should be there.
Physiotherapy and rehabilitation Physiotherapy with regular muscle exercise would help in preventing contracture formation and maintaining the muscle strength as much as possible according to the severity. Rehabilitation would help in carrying out day to day activities and mobilizing in disabled patients. Regular physiotherapy would also help in maintaining bone integrity as these patients are highly vulnerable for osteoporosis due to long term steroids. Any how the vigorous training should be better avoided. Physiotherapy and rehabilitation
Physiotherapy with regular muscle exercise would help in preventing contracture formation and maintaining the muscle strength as much as possible according to the severity. Rehabilitation would help in carrying out day to day activities and mobilizing in disabled patients. Regular physiotherapy would also help in maintaining bone integrity as these patients are highly vulnerable for osteoporosis due to long term steroids. Any how the vigorous training should be better avoided.
Measures to prevent Gastro Oesophageal Reflux Disease The patient should take light meals and the dinner should be adequately apart from the sleeping time. The head end should be little propped up. Proton pump inhibitors and anti emetics can be used if persistently symptomatic. Measures to prevent Gastro Oesophageal Reflux Disease
The patient should take light meals and the dinner should be adequately apart from the sleeping time. The head end should be little propped up. Proton pump inhibitors and anti emetics can be used if persistently symptomatic.
Dietary modification The patients should take a balanced diet and a high protein diet should be recommended if there is a significant loss. Dietary modification
The patients should take a balanced diet and a high protein diet should be recommended if there is a significant loss.

Management - Specific

Fact Explanation
Drug management for the muscle disease Mainstay of drug management for the muscle disease is corticosteroids, usually prednisolone. It should be slowly tapered once started to prevent the relapses. Occurrence of side effects should be monitored; peptic ulcers, osteoporosis, cushing's syndrome, diabetes mellitus etc and treated accordingly. Steroid sparing drugs also can be used when the steroid induced side effects are significant. Those steroid sparing drugs are Methotrexate, Azathioprine, Cyclophosphamide, Cyclosporine, Mycophenolate mofetil, Leflunomide and Chlorambucil.
Other than that monthly high doses of IV immunoglobulin is beneficial in resistant cases. Monoclonal antibodies like rituximab are also shown to have benefits.
Drug management for the muscle disease
Mainstay of drug management for the muscle disease is corticosteroids, usually prednisolone. It should be slowly tapered once started to prevent the relapses. Occurrence of side effects should be monitored; peptic ulcers, osteoporosis, cushing's syndrome, diabetes mellitus etc and treated accordingly. Steroid sparing drugs also can be used when the steroid induced side effects are significant. Those steroid sparing drugs are Methotrexate, Azathioprine, Cyclophosphamide, Cyclosporine, Mycophenolate mofetil, Leflunomide and Chlorambucil.
Other than that monthly high doses of IV immunoglobulin is beneficial in resistant cases. Monoclonal antibodies like rituximab are also shown to have benefits.
Drug management for the skin disease The treatment of dermatological lesion is often difficult. Anti malarials such as hydroxychloroquine and chloroquine are shown to be beneficial. When those are contraindicated methotrexate can be used. Other than that IV immunoglobulin, Sirolimus, dapsone and rituximab also has benefit on skin lesions. Drug management for the skin disease
The treatment of dermatological lesion is often difficult. Anti malarials such as hydroxychloroquine and chloroquine are shown to be beneficial. When those are contraindicated methotrexate can be used. Other than that IV immunoglobulin, Sirolimus, dapsone and rituximab also has benefit on skin lesions.
Management of calcinosis Early treatment of myositis will reduce the complication, calcinosis. Calcium channel blockers such as diltiazem has place in gradual resolution of the calsinosis. Established calcinotic nodules can be removed surgically. Management of calcinosis
Early treatment of myositis will reduce the complication, calcinosis. Calcium channel blockers such as diltiazem has place in gradual resolution of the calsinosis. Established calcinotic nodules can be removed surgically.
Management of arrhythmia and cardiac failure Anti arrythmetics according to the type of arrhythmia has to be tried. For the cardiac failure spironolactone, ACE inhibitors and beta blockers can be used. Management of arrhythmia and cardiac failure
Anti arrythmetics according to the type of arrhythmia has to be tried. For the cardiac failure spironolactone, ACE inhibitors and beta blockers can be used.

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