A middle-aged woman presented with generalized weakness, rashes, and sores. On examination, she had hyperpigmentation, edema, and rashes on her buttocks and groin. Tests found elevated muscle enzymes. She was diagnosed with dermatomyositis and admitted for IV fluids and steroids. Her symptoms worsened, and she developed difficulty raising her arms, confirming the diagnosis of dermatomyositis. She was started on immunosuppressants and referred for palliative care.

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. 