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DERMATOMYOSITIS Your condition is called dermatomyositis.

This is a
17 YEAR OLD GIRL CAME TO YOUR CLINIC BECAUSE OF connective tissue disorder characterized by weakness
EDEMA AT THE DORSUM OF YOUR FINGERS AND RASH. and inflammatory changes in your muscle and skin.
TASK: TAKE A HISTORY, PE FINDINGS FROM EXAMINER, - Dermato – means skin, Myo – means muscle –
DIAGNOSIS AND MANAGEMENT OF THE CASE Citis –inflammation
(little boy, early adolescent, or late age, if late age – but - Put them together – skin and muscle
prognosis – underlying malignancy) inflammation
We will do some investigation to confirm your diagnosis
History: and to check the gravity of the disesase.
Would you tell me more about your problem.. We have to screen some underlying disease.
When did it start? For 2-3months I first noticed that my We will do CK (very high) - specific, rheumatic factor
hand starts to swell, It first started with one finger (+), ANA (+) –not specific
Does it hurt in the muscles or joints? + pain in the joint Electromyogram – inflamed muscle, inflammatory
Did you take any medications? I took some panadol changes in the muscle
sometimes it helps sometime not Muscle biopsy – find muscle necrosis – may become
How about the rash? When did it start to appear fibrosed, contracture – discuss physiotherapy
After 2- 3weeks they start to appear Do MRI of affected muscles – to detect the myositis –
Any joint pain in any parts of the body? Sometime my definitive diagnosis
knee hurt me Screening test for other underlying disease:
Any rash in other parts of the body? Face (eyelids), v CXR,
shape area of the chest (because it is exposed to the Breast ultrasound (Mammography)
sun, upper limbs, knee, back of the elbow. tumour markers,
Ask about muscles – what about your shoulder and hip abdominal and pelvic ultrasound,
area, do you have any pain? I have pain in my shoulder Urine analysis
muscle, sometime I feel that I could not feel my
shoulder and sometime I feel that I am carrying my Predinisolone tablet 40-60 mg
shoulder (Weakness) Monitor muscle enzymes and CK – improve early.
Do you feel that you are hot to touch? Decreased
Nausea vomiting? Decrease dose to 10-15mg
How is your appetite?
Did you have changes in weight? +weight loss If this medication will not help her – we can give
immunosuppressive drugs like methotrexate
Lung, breast pancreas, ovaries, lymph nodes –
malignancy related (JM Offer her splint of the affected muscle
Any unexplained symptoms with your chest – Refer to rheumatologist and physiotherapist
Do you cough out blood?
Do you have any breast pain or lump? Bad prognosis – for elderly patients.
Any abdominal pain?
Do you smoke or drink alcohol Look for photo of the rash
How about your periods? p.287 John Murtagh
Are you sexually active or not?
Do you use any contraception?
Family history
Medication and allergies
Waterworks, Bowel

PE: GA: looks tired, weak in pain


She has rash – erythamatous,scaly rash mauve or
purple in colour
upper eyelid, elbow, knuckles and the back of the hand
and knees as well
VS: + low grade fever
Joint examination: + arthralgia + pain in shoulder
muscle
Neurological Examination of upper and lower limb +
weakness, decrease in power and tone of the proximal
muscles of the upper limb

Explanation: (Dermatomyositis, Polymyalgia rheumatica


– no rash, pain in shoulder)
Polymyositis
and dermatomyositis
Polymyositis is an uncommon systemic disorder whose
main feature is symmetrical muscle weakness and
wasting involving the proximal muscles of the shoulder
and pelvic girdles.

Clinical features

 Any age group


 Peak incidence 40–60 years
 Female to male ratio = 2:1
 Muscle weakness and wasting proximal limb
muscles
 Main complaint is weakness
 Muscle pain and tenderness in about 50%
 Arthralgia or arthritis in about 50% (resembles
distribution of rheumatoid arthritis)
 Dysphagia in about 50% due to oesophageal
involvement
 Raynaud's phenomenon
 Consider associated malignancy: lung and
ovary Figure 32.4 Clinical features of
polymyositis/dermatomyositis
DxT: weakness + joint and muscle pain + violaceous
facial rash = dermatomyositis Diagnosis
The rash  Muscle enzyme studies (serum creatine kinase
and aldolase)
The distinctive rash shows features of photosensitivity.  Biopsies—skin and muscle
There is violet discolouration of the eyelids, forehead  EMG studies—show characteristic pattern
and cheeks, and possible erythema resembling sunburn
and periorbital oedema. There is a characteristic rash
Treatment includes corticosteroids and cytotoxic drugs.
on the hands, especially the fingers and nail folds. The
Early referral is appropriate.
knees and elbows are commonly involved.

The main features are summarised in Figure 32.4.

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