DERMATOMYOSITIS

Dermatomyositis is a systemic inflammatory disease of striated muscles, skin

and joints; less frequently the internal organs are involved. If the skin is not
affected, the disease state is called polymyositis.
The disease is characterized by a progressive severe necrotic myositis with
predominant involvement of the muscles of the proximal parts of the limbs. As the
disease progresses, the muscular tissue undergoes atrophy and is replaced by
fibrous tissue. Similar processes occur in the myocardium. The parenchymal organs
are affected by dystrophy; the vessels of the muscles, internal organs and skin are
also involved in pathology.
Aetiology. Two forms of dermatomyositis are distinguished: idiopathic and
secondary tumour. The aetiology of idiopathic dermatomyositis and other diseases
of this group is unknown. But the factors promoting the onset (and later
exacerbation) of the disease are: (1) exposure to the sun; (2) cooling; (3) infection
(acute respiratory disease, influenza, acute pharyngitis, etc.); (4) endocrine
reconstruction (climax, pregnancy, labour); (5) emotional stress; (6) injury,
surgical operation; (7) sensitiza-tion with drugs (aminazine, insulin, antibiotics); (8)
vaccination; (9) contact with epoxy resins, photographic developers; (10)
physiotherapeutic procedures.
The hereditary and genetic factors are probably also involved- the
histocompatibility antigen B-8 is detected in the patients.
Tumour (secondary) dermatomyositis makes 25 per cent of malignant tumours.
Dermatomyositis most frequently occurs in patients with cancer of the lungs,
intestine, prostata, ovaries, and haemoblastosis. Development of
dermatomyositis in patients aged over 40 is almost always associated with
tumours.
Pathogenesis. The immune response becomes upset by the action of virus and the
genetic factor (in the presence of the predisposing factors) or the action of tumour
antigens. The system of B- and T-lymphocytes becomes upset: antibodies to
skeletal muscles are produced, and the T-lymphocytes become sensitized to them.
The antigen-antibody reaction and the cytotoxic effect of T-cells sensitized to muscles
promote formation of immune complexes and their deposition in muscles and the
microcir-culatory vessels of various organs. Elimination of immune complexes
releases lysosome enzymes and provokes immune inflammation of the muscles and
internal organs. New antigens are released during inflammation which promote
further formation of immune complexes and conversion of the disease into a chronic
pathology and involvement of intact muscles. The pathogenesis of dermatomyositis
is shown schematically in Diagram 27.

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 Clinical picture. The manifestations of the disease are systemic and characterized
by the presence of many syndromes. The main syndromes are: (1) muscular
(myositis, muscular atrophy, calcification); (2) cutaneous (erythema, oedema of the
skin, dermatitis, pigmentation and depigmenta-tion, telangiectasia, hyperkeratosis,
erythema nodosum, urticaria); (3) articular (arthralgia, involvement of periarticular
tissues, true arthritides are rare); (4) visceral (myocarditis, cardiosclerosis;
pneumonitis, aspiration pneumonia, pulmonary fibrosis, gastritis, enterocolitis,
gastrointestinal haemorrhage; glomerulonephritis with chronic renal failure,
'myoglo-binuric' kidney with acute renal failure, polyneuropathy).
The periods of the disease are:
Period I (initial)—lasts from several days to a month and longer; manifested
only by muscular and/or cutaneous symptoms;
Period II (advanced)—the clinical picture of the disease is manifest;
Period III (terminal)—manifested by dystrophy of the internal organs and
symptoms of their severe dysfunction; complications develop during this period.

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 Three forms of the disease course are distinguished:
acute: generalized affection of the skeletal muscles develops to immobilize the
patient completely; the muscles of the throat ring and oesophagus are involved
progressively (dysphagia, dysarthria); the internal organs (especially the heart) are
rapidly affected and the patient dies in 2-6 months after the onset of the disease;
subacute: the symptoms develop more slowly and gradually; severe affections of
the muscles and organs develop in 1-2 years;
chronic: the disease runs a prolonged cyclic course; atrophy and sclerosis
prevail; local affection of muscles is possible.
The clinical picture of dermatomyositis is thus quite polymorphous: different
information can therefore be obtained during one and the same stage of diagnostic
examination.
The first stage of diagnostic examination reveals the character of the disease
onset: acute (elevation of body temperature to 38-39°C, erythema of the skin and
pain in the muscles) or gradual (moderate weakness, mild myalgia and arthralgia,
which intensifies after exercise, exposure to the sun or other adverse effects).
The most specific complaints are due to muscular involvement: the patient
complains of weakness, he or she is unable to sit down or stand up unassisted, climb
or ascend stairs; the patient often suffers from muscular pain. Myasthenia and
myalgia are usually located in the proximal parts of the extremities, and also in the
spine and neck.
If the throat muscles are involved, the patient complains of difficult swallowing;
liquid food can pass back through the nose. The voice is nasal and hoarse due to
affection of the laryngeal muscles. Diplopia is due to affection of the ocular muscles;
urinary and faecal incontinence is due to dysfunction of the sphincters.
The patient notes persistent change in the colour of the skin at sites exposed to the
sun (face, hands, neck), and also on the lateral surfaces of the thighs and shins.
Lilac-coloured periorbital oedema is characteristic. If the mucosa is affected, the
patient complains of dryness and burning pain in the eyes, the absence of tears.

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 Involvement of various organs is manifested by the symptoms characteristic of
myocarditis, cardiosclerosis, pneumonitis, glomerulonephritis, polyneuritis, arthritis,
etc.
Information about previous treatment characterizes its efficacy and, indirectly, the
character of the disease course: previous use of amino-quinoline preparations
suggests chronic disease; administration of pred-nisolone and cytostatics suggests a
more acute course of the disease.
During the second stage of diagnostic examination of patients with a manifest
clinical picture of the disease, muscular affections become first of all apparent: the
muscles are firm and flexid, their volume is increased, palpation is painful. If the
mimic muscles are involved, the facial expression may be lost. The muscles are later
affected by atrophy, which is especially marked in the shoulder girdle. Palpation of
the muscles can reveal local induration—calcification of the muscles and
subcutaneous fat. Calcinosis is more common in the young with diffuse affection of
the muscles during transformation of acute disease to its subacute or chronic form.
The body weight sometimes decreases by 10-20 kg.
Cutaneous affections are not obligatory for dermatomyositis, but their presence on
the open parts of the body is manifested by oedema, erythema, dermatitis, sometimes
petechial eruptions, haemorrhages, telangiectasia. The lilac-coloured periorbital
oedema is typical. Erythema is quite stable (with a cyanotic hue) and attended by
itching and scaling. The skin is often dry and the nails brittle; the hair falls out.
The physical signs of visceral affections in dermatomyositis, like in systemic
scleroderma, are not very marked compared with those in systemic lupus
erythematosus. A certain disagreement can be seen between the manifestation of the
pathomorphological changes in the organs and their clinical manifestations.
Involvement of the heart (myocarditis, cardiosclerosis) are manifested by non-
specific symptoms such as cardiac enlargement, dull tones, tachycardia and
arrhythmia (extrasystole). Marked changes in the myocardium can evoke symptoms
of heart failure.

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 Involvement of the lungs in the form of pneumonitis is not obvious. Fibrosis is
manifested by emphysema of the lungs and respiratory insufficiency. Aspiration
pneumonia is characterized by all specific physical symptoms.
Gastrointestinal involvement is characterized by dysphagia: solid food would be
regurgitated while liquid food passed back through the nose. Involvement of the
gastrointestinal vessels can cause gastrointestinal bleeding attended by all specific
symptoms of this complication. Moderate enlargement of the liver is seen in some
patients; the hepatolineal syndrome with enlargement of the lymph nodes is less
common.
Renal involvement (glomerulonephritis) can cause arterial hypertension with
subsequent hypertrophy of the left ventricle
The neurological symptoms are manifested by changed sensitivity: peripheral or
radicular hyperaesthesia, hyperalgesia, paraesthesia and areflexia.
During the third stage of diagnostic examination use is made of methods that
help assess acuity of inflammation and degree of muscular involvement.
The acuity of the process can be assessed by non-specific acute-phase signs
(accelerated ESR, high fibrinogen and C-reactive protein, hyper-α 2-globulinaemia)
and the signs of immunologic shifts (presence of the rheumatoid factor, increased
content of gamma-globulins, antibodies to nucleoprotein and soluble nuclear
antigens; idiopathic dermatomyositis is manifested by increased IgG).
The degree of muscular involvement is characterized by some biochemical
signs. The activity of serum enzymes (AST, ALT, CPK, aldolase) is high; the
creatine to creatinine ratio increases due to excretion of creatine with the urine and
decreased creatininuria. Marked muscular affection is manifested by myoglobinuria.
Tumour associated with dermatomyositis is characterized by increased alphaj
lipoprotein fraction in the blood serum.
Electromyography is important for the diagnosis of muscular affections. This
method reveals normal electric activity of the muscles during spontaneous relaxation;
voluntary contractions are characterized by a low amplitude.

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 Biopsy of the skin and muscles reveals severe myositis with the loss of the striated
structure of the fibres, with fragmentation, granular or waxy degeneration, necrosis,
lymphoid-plasma cell infiltration and fibrosis.
Other methods (ECG, x-ray, endoscopy) are necessary to assess the condition of
the involved internal organs and to detect tumour (in suspected malignancy).
Diagnosis. Major and minor symptoms of the disease should be taken into
consideration:
Major symptoms:
(1) muscular syndrome (diagnosed clinically);
(2) cutaneous syndrome (the periorbital oedema included);
(3) histological signs of muscular affections (loss of the striated struc
ture, necrosis, degeneration, lymphoid-plasma cell infiltration, atrophy,
fibrosis);
(4) EMG changes (normal electric activity during muscle relaxation and
low-amplitude during voluntary contractions);
(5) increased enzyme activity (by 50 per cent and more) indicating
necrotic changes in the muscles.
Minor symptoms:
(1) muscle calcification;
(2) visceral-muscular syndrome (dysphagia, due to involvement of the throat and
oesophageal muscles).
The diagnosis of dermatomyositis can be considered trustworthy in the presence
of: (a) any three major symptoms; (b) combination of two major and two minor
symptoms.
The diagnosis is only probable in the presence of: (a) isolated cutaneous syndrome;
(b) combination of two major symptoms; (c) combination of one major and one
minor symptoms.
Dermatomyositis should be differentiated from infectious and neurologic
diseases, systemic scleroderma, systemic lupus erythematosus, and rheumatoid
arthritis. The differentiation is based on the following:
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 1.Persistance of the articular syndrome in rheumatoid arthritis, detection of
erosion on the joint surfaces by x-ray, the absence of specific dermatomyositis
changes in the skin and muscles.
2.As distinct from systemic lupus erythematosus, visceral pathology in
dermatomyositis is not so pronounced and occurs less frequently; the clinical
picture of dermatomyositis is dominated by muscular affections; laboratory
findings (immunologic signs in particular) are changed to a smaller degree.
3.As distinct from systemic scleroderma, cutaneous affections in dermatomyositis
are quite different; the specific changes in the hands or Raynaud's phenomenon
are absent; the muscular syndrome (severe myasthenia included) is the leading
symptom. Nevertheless, differential diagnosis of systemic lupus erythematosis and
dermatomyositis is very difficult. Electrophysiological and morphological methods
of examination are necessary in complicated cases.
4.If the course of dermatomyositis is acute, it is necessary to rule out infection
(sepsis, erysipelas, etc.), which is only possible in follow-up observation of the
patient.
5.If adynamia prevails and reflexes are upset, it is necessary to differentiate the
disease from neurologic pathology. This can be accomplished by observation of the
patient by the internist and neuropathologist.
A complete clinical diagnosis of dermatomyositis should include the following:
(1) the period of the disease; (2) form of the disease course; (3) clinico-
morphological characteristic of system and organ involvement with indication of the
leading syndromes and the presence (absence) of dysfunction of the involved organs
or systems.
Treatment. The main object of treatment is to suppress the activity of immune
reactions and immune inflammation, and to normalize the function of severely
involved organs and systems.
The best effect is attained with glucocorticoids. Prednisolone should be preferred.
The doses and duration of the therapy depend on the acuity of the process and the
clinical effect of the therapy. Acute disease should be treated with 80-100 mg/day

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doses; subacute—-60 mg/day; exacerbations should be managed by 30-40 mg/day
doses.
Prednisolone should be given for 2-3 months and longer, until a pronounced
clinical effect is attained. The dose should then be slowly decreased to maintenance
doses: 30-40 mg/day during the first year of the disease (treatment being started in
the acute or subacute period), and 10-20 mg/day during the 2nd-3rd year of the
disease. The therapy can be suspended in stable remission (in the presence of its
clinical and laboratory signs).
If prednisolone fails, or it is not tolerated by the patient (complications),
cytostatics should be given. Azathioprine is popular: 1-3 mg per kg body weight for
2-6 months. The dose can be decreased if the preparation is given together with
prednisolone. Cytostatics are given for 2-6 months and the dose is then decreased.
Aminoquinoline preparations (delagil, plaquenil) are given in the following cases:
(1) in chronic disease without signs of activity;
(2) if prednisolone or cytostatic doses are decreased to lessen the risk of possible
complications.
Additional methods:
(1) if the painful articular syndrome prevails, non-steroidal anti-
inflammatory preparations are given: indomethacin, voltaren, brufen;
(2) if prednisolone is given for a long time, potassium preparations and anabolic
steroids (nerobol, retabolil) should be prescribed;
(3) calcinosis should be managed by complexons (EDTA, disodium salt), which
form complex compounds with calcium ions to promote their excretion with the
urine;
(4) patients with chronic dermatomyositis without signs of activity should be
given physiotherapy: paraffin applications, electrophoresis with hyaluronidase,
therapeutic exercise.
Prognosis. With incorporation of prednisolone and cytostatics into treatment of
acute and subacute forms of the disease, the prognosis has markedly improved. If the
disease transforms into a chronic pathology, the patient can regain his working

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capacity. The prognosis of secondary (tumour) dermatomyositis depends on
efficacy of operative treatment. If the operation is successful, all signs of the disease
can abate.
Prophylaxis. Prevention of exacerbations (secondary prophylaxis) can be attained
by maintenance therapy, treatment of infection foci, and by general invigorating
treatment. The patient's relatives should be given primary prophylaxis: ruling out
overexertion, excessive exposure to the sun, or cooling.

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