Dermatomyositis

By : Mariam Qais Hatef – 5th Stage

Supervised by : Dr.Sabeeh Almashhadani

2020
 Introduction:

Dermatomyositis (DM) is a rare type of

acquired inflammatory myopathy(group of
diseases that involve chronic muscle
inflammation and weakness.)
characterized by inflammatory and
degenerative changes of the muscles and
skin. (1)

Epidemiology:
The incidence of DM peaks at ages 40–50, but the disease can
affect people of all ages, it tends to affect more women than men.
(2) 

Causes:
The exact cause of dermatomyositis isn’t known. However, it has
many similarities to an autoimmune disease, anyone can develop
dermatomyositis, it may result from an initial viral infection or
cancer, either of which could raise an autoimmune response. (3)

Signs and Symptoms:

The main symptoms include several kinds of skin rash along
with muscle weakness in both upper arms or thighs. (4)

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1. Cutaneous features (5)
In many patients, the first sign of dermatomyositis is the presence
of asymptomatic, itchy or burning rash [Figure-1]. The rash often,
but not always, develops before the muscle weakness.

Purple eyelids, which are described as Violaceous rash affecting cheeks,

heliotrope, as they resemble the nose, shoulders, upper chest and
heliotrope flower. elbows. Mostly sun-exposed areas.

Purple papules or plaques are found on A scaly scalp and thinned out hair
bony prominences, especially the may occur.
knuckles (Gottron papules).

Figure 1 : DM types of rash.

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2. Myositis (5)
Muscle weakness may arise at the same time as the
dermatomyositis rash, or it may occur weeks, months or years
later. Proximal muscles are affected (upper arms, thighs). The
first indication of myositis is when the following everyday
movements become difficult :

 Climbing stairs or walking

 Rising from a sitting or crouching position

 Lifting objects

 Raising arms above the shoulders, such as when combing hair

 Difficulty swallowing (dysphagia)

Occasionally the affected muscles ache and become tender to

touch.

Diagnosis:
The following tests usually confirm the diagnosis of DM (6) :

• Creatine kinase (CK) and sometimes aldolase, aspartate

aminotransferase (AST) and lactic dehydrogenase (LDH).

• Non-specific antinuclear antibody (ANA) is found in most

patients, specific Anti-Mi-2 is found in one quarter and Anti-Jo-1
in a few.

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 • Skin biopsy of the rash: the microscopic appearance of an
interface dermatitis is similar to systemic (acute) lupus
erythematosus

• Biopsy of an affected muscle

• Electromyography (EMG) testing

• Magnetic resonance imaging (MRI) scan of muscles

Treatment:
There's no cure for dermatomyositis, but treatment can improve
skin and muscle symptoms, including (7) :

 Corticosteroids : such as prednisone .

 Corticosteroid-sparing agents : The two most common are

azathioprine and methotrexate

 Rituximab is an option if initial therapies don't control your

symptoms.

 Antimalarial : (hydroxychloroquine) for a persistent rash.

 Sunscreens and wearing protective clothing and hats.

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 References :
1. Jeffrey S. Orringer., Amy J. McMichael., David J. Margolis., Alexander H.
Enk., Anna L. Bruckner., Masayuki Amagai. and Sewon Kang.,
n.d. Fitzpatrick's Dermatology, 9E. 9th ed , ch 62 .
2. Callen, J. and Wortmann, R., n.d. Dermatomyositis. Clinics in Dermatology,
24(5), pp.363-373.
3. Dermnetnz.org. 2020. Dermatomyositis | Dermnet NZ. [online] Available at:
<https://dermnetnz.org/topics/dermatomyositis/> [Accessed 21 August 2020].
4. Hogan Smith, K., 2017. Review of Rare Diseases Resources: National
Organization for Rare Disorders (NORD) Rare Disease Database, NIH Genetic
and Rare Diseases Information Center, and Orphanet. Journal of Consumer
Health on the Internet, 21(2), pp.216-225.
5. Mayoclinic.org. 2020. Dermatomyositis - Diagnosis And Treatment - Mayo
Clinic. [online] Available at: <https://www.mayoclinic.org/diseases-
conditions/dermatomyositis/diagnosis-treatment/drc-20353192> [Accessed 21
August 2020].
6. NORD (National Organization for Rare Disorders). 2020. Dermatomyositis -
NORD (National Organization For Rare Disorders). [online] Available at:
<https://rarediseases.org/rare-diseases/dermatomyositis/> [Accessed 21 August
2020].
7. Sudoł-Szopińska, I., Larkman, N., O'Connor, P., Cotten, A. and Jacques, T.,
2018. Musculoskeletal Manifestations of Non-RA Connective Tissue Diseases:
Scleroderma, Systemic Lupus Erythematosus, Still's Disease,
Dermatomyositis/Polymyositis, Sjögren's Syndrome, and Mixed Connective
Tissue Disease. Seminars in Musculoskeletal Radiology, 22(02), pp.166-179.
8. Figure 1 : Dermnetnz.org. 2020. Dermatomyositis | Dermnet NZ. [online]
Available at: <https://dermnetnz.org/topics/dermatomyositis/> [Accessed 21
August 2020].