Case Management and Brief Literature Review of Dermatomyositis

1. Dr. Bernard Fiifi Brakatu

2. 
 PC: rash, general weakness, multiple sores,
insomnia – 6/12
 HPC: Pt had been well until the Sxx began and
progressed gradually until she presented at the
hospital
 ODQ: Wt loss+, Easy fatigability+, dizzinesso
 SE: cougho, chest paino, palpitationso,
dysphagia+(with solid food), odynophagia+,
vomitingo, diarrheao, dysuriao, frequencyo,
headacheo

3. 
 PMH – HPTo, DMo
 DH – Nil of note
 SH – Trader, ROHo, Smokingo
Hx

4. 
 Chronically ill middle-aged woman with generalized
hyperpigmentation of the skin
 Po, Jo, Fo, Hyd-sat, Pedal Edema+, Facial puffiness+
 CVS – S1S2Mo PR 90bpm RGV, BP 130/80mmHg
 Chest – clinically clear
 CNS – Fully conscious and alert
 S/L- Generalized Rash with Multiple ulcers at the
gluteal region and at the groin
O/E

5. 
?SLE
?RVI
Diagnosis

6. 
 Admit to GFW
 FBC, BUE/Cr, LFTs, VCT, ANA, Anti-dsDNA
 IVF – 1L R/L, 1L 5%Dex + Pabrinex I &II
 IV Lasix 40mg bd x 48hrs
 Fluid input/output chart
 Daily wound dressing
 Ted Stockings
 Turn regulartly in bed(12hrly)
Plan

7.  Day 3- Tb Flucloxacillin and Z9inc Oxide cream
added
 Day 4 – Tb Prednisolone 40mg dly x 7
 Day 6 – VCT- non-reactive, Physiotherapy
 Day 10 – dysphagia worsening; ?scleroderma
 Day 11 – abd pain; steroid-induced gastritis;
omeprazole and nugel added
 Day 12 – pt unable to elevate arms above the
head(proximal myopathy); Dermatomyositis; Tb
Azathioprine 50mg dly x 7, Tb Prednisolone 50mg
dly x 17/7, Gynae review for malignancy
 Day 12 –date – Palliative/ Physiotherapy
R

8. 
LITERATURE REVIEW

9. 
 Dermatomyositis(DM) is a rare autoimmune
disorder characterized by inflammation of striated
muscle, causing proximal muscle weakness with
skin involvement.
 NB: Polymyositis has the same clinical features but
has no skin involvement.
Definition

10. 
 Incidence is about 2-10/million population per
annum
 It occurs in all races and at all ages
 M:F ratio is 1:3
 Etiology is unknown, although viruses(e.g. Coxsackie,
rubella, influenza) have been implicated
 Persons with HLA-B8/DR3 appear to be
predisposed
Epidemiology and
Etiology

11. 
 The onset can be either insidious, spanning over
months, or acute
 General malaise, weight loss and fever can develop
in the acute phase
 There is myalgia, polyarthritis and Raynaud’s
phenomenon
 Shoulder and pelvic girdle muscles become wasted
but are not usually tender and so squatting and
climbing of stairs become difficult
 Face and distal limb muscles are not usually affected
Clinical Features

12. 
 As the disease progresses, pharyngeal, laryngeal and
respiratory muscle involvement can lead to
dysphonia and respiratory failure
 These complications are rare if the disease is treated
early.
C/F

13. 
 Distinguish DM from PM with characteristic rash
affecting the eyelids where heliotrope (purple)
discoloration is accompanied by periorbital oedema,
and the fingers where one sees purple-red raised
vasculitic patches
 These patches occur over the knuckles (Gottron’s
papules) in 70% of patients, and this appearance is
highly specific for DM
 Ulcerative vasculitis and calcinosis of the
subcutaneous tissue occurs in 25% of cases. In the
long term, muscle fibrosis and contractures of joints
occur.
C/F

14. 
Gottren’s papules

15. 
Gottren’s papules

16. 
 Pulmonary interstitial fibrosis, Raynaud’s phenom-
enon, arthritis and hardening and fissuring of skin
over the pulp surface of the fingers (mechanic’s
hands)
 Dysphagia is seen in about 50% of patients owing to
oesophageal muscle involvement.

17. 
 The relative risk of cancer is 2.4 for male and 3.4 for
female patients, and a wide variety of cancers have
been reported usually gynaecological and carcinoid
tumors
Association with
Malignancies

18. 
 Serum creatine kinase (CK), aminotransferases,
lactate dehydrogenase (LDH) and aldolase are
usually raised
 ESR and CRP may be raised.
 Serum autoantibody studies : ANA is usually
positive in people with DM. Rheumatoid factor is
present in up to 50%
 Electromyography(EMG): shows changes consistent
with myositis
Investigations

19. 
 MRI- shows abnormally inflamed muscle
 Needle muscle biopsy
 Screening for malignancy - CXR, mammography,
pelvic/abdominal ultrasound, urine microscopy and
tumor markers, PET scan

20. 
 Bed rest and physiotherapy
 Prednisolone is the mainstay of treatment – 0.5-
1.0mg/kg/body wt as initial therapy continued
until at least 1 month after myositis has become
clinically and enzymatically inactive
 Early intervention with steroid-sparing agents such
as meth- otrexate, azathioprine, ciclosporin,
cyclophosphamide and mycophenolate mofetil is
common, especially where there is clinical relapse or
rise in CK as the dose of steroids is reduced
 Taper steroids slowly
Treatment

21. 
 Intravenous immunoglobulin therapy (IVIG) is
helpful in some refractory cases
 Monoclonal antibodies such as Rituximab may be
helpful
 Counsel patient
 Review once to twice a year

22. 

23. 