Dermatomyositis

DEFINITION

 Dermatomyositis (DM) continues to be classified as a

form of myositis and considered one of the idiopathic
inflammatory myopathies. Characterized by
inflammation and damage to the skin and muscle.
 Patients who have the characteristic rash but no clinical
signs or symptoms of myositis defined as clinically
amyopathic dermatomyositis (CADM)
 EPIDEMIOLOGY

• DM has an onset occurring at two peaks, at 5 to 14

years and 45 to 64 years of life.
• The disease affects women two to three times more
than often than men
• Incidence rates ranging from 5 to 10 per 1 million per
year and prevalence estimates of 10 to 20 per 100,000
 CLINICAL FEATURES
• Cutaneous Finding
 The cutaneous manifestations are typified by violaceous
erythema in many sites, most notably the eyelids, upper
chest, back, elbows, knees, and lateral hips in addition to
proximal nailfold capillary dilation and pericapillary
hemorrhage.
 Trunk involvement is often seen on the posterior neck, upper
back, and shoulders, known as the shawl sign
 The heliotrope sign may exemplify the pink to purple violet
hue of the eruption, resembling the color of the flower petals.
 Gottron sign is symmetric macular violaceous erythema
over the IP joints, olecranon processes, patellae, and
medial malleoli. The violaceous to pink papules over the
IP and MCP joints are termed Gottron papules
 The violaceous erythema and poikiloderma on the lateral
hips and lateral thighs is termed the Holster sign
 Proximal nailfold involvement can present as subtle
edema or erythema as well as microscopic or clinically
obvious capillary dilation.
EXTRACUTANEOUS FINDINGS
• Pulmonary Involvement
 ILD is the most common pulmonary manifestation in DM and is a leading cause of
morbidity and mortality in these patients. Others include aspiration pneumonia; drug-
induced pneumonitis; and, rarely, pulmonary hypertension
• Muscle Involvement
 Myositis in DM typically presents as symmetrical proximal muscle weakness. Others
may be present myalgia, dysphonia, dysphagia.
• Joint Involvement
 Arthralgias and arthritis
• Gastrointestinal Involvement
 Abdominal pain, the sequelae include ulceration and perforation
• Cardiovascular Involvement
 Myocarditis and cardiomyopathy
• Malignancy
 ETIOLOGY

Environmental trigger:
• UV exposure
• infection
• malignancy

 On the cellular level, these triggers could result in antigen

modification or upregulation, cellular death, and activation of
nucleic acid response→ increased production of IFN,
endoplasmic stress, and activation of the innate immune system.
 PATHOGENESIS

• Innate immune system

 Increased production of IFN-α/β–inducible proteins by
plasmacytoid dendritic cells and myocytes can also result in
endothelial damage
 Activation of the innate immune system→high levels of interferon
(IFN)-induced genes and proteins found in blood, muscle and
skin→induce DM autoantigens (eg, MDA5)→ activate immature
dendritic cells to become effective antigen-presenting cells, and
upregulate major histocompatibility (MHC) class I expression, and
activate lymphocytes→activating cytotoxic effector cells
• Adaptive immune system
 Certain HLA alleles are clearly some of the greatest
risk factors for the development of DM. The HLA-B8
allele was the first allele found in increased prevalence
in 12 of 17 (75%) juvenile patients
 Human leukocyte antigen (HLA)→ implicating
activation of T cells
• Nonimmune system
 The endoplasmic reticulum stress response that may be partially
responsible for some of the weakness symptoms seen in
DM→upregulation of MHC class I or other proteins→ activation of
inflammatory nuclear factor kappa B (NF-κB) signaling
pathways→mitochondrial dysfunction, and elevated reactive oxygen
species, all of which can contribute to weakness

 Muscle enzyme drops as myositis continues →perifascicular

muscle atrophy and fibrosis
 DIAGNOSIS

Helpfull Skin findings

 microscopic periungual telangiectasias
 lateral digit hyperkeratosis
 scalp erythema
 dysesthesia
 “red-on-white” patches
 the ovoid palatal patch
 grossly visible periungual telangiectasias
 Gottron papules
Muscle Disease
 Myositis is not a requirement for the diagnosis but helpful when a patient
presents with a suspicious rash
Muscle involvement:
• Dysphagia, dysphonia, and myalgia
• Weakness on examination
• Elevation of muscle enzymes
• EMG,MRI and muscle biopsy in cases in which a cause for muscle
symptoms is still not clear.
Syndromic Presentation
 The presence of the more “typical” cutaneous features of DM:
• Dysphagia, dysphonia, and myalgia
• Weakness on examination
• Elevation of muscle enzymes
• EMG,MRI and muscle biopsy in cases in which a cause for muscle symptoms is still not
clear.

 consider anti-MDA5 DM if a patient presenting with:

• alopecia,
• cutaneous or mucosal ulceration
• palmar erythematous papules,
• severe arthralgia or arthritis
• shortness of breath,
Diagnostic Laboratory Evaluation
1. AUTOANTIBODIES
 ANA testing should be done using method of direct immunofluorescence. ANA test results
can be negative in DM 50% of the time but are usually positive in systemic lupus
erythematosus

 Myositis-specific autoantibodies (MSAs) associated with DM

• TIF1-γ
• NXP2
• MDA5
• Small ubiquitin-like modifier activating enzyme (SAE)
• Mi-2
• Jo-1
• the other antisynthetase antibodies (PL-7, PL-12, EJ, OJ, SRP)
2. MUSCLE ENZYMES
 Bio-markers of muscle inflammation:
• creatine kinase
• aldolase
• lactate dehydrogenase [LDH]
• aspartate aminotransferase [AST]
• alanine aminotransferase [ALT]

3. EMG
 Myositis may be detectable on elec_x0002_tromyographic studies in 70% to 90% of patients
with DM with active muscle disease and the sensitivity to detect myositis decreases over
time

4. MRI
Pathologi
DIFFERENTIAL DIAGNOSIS
 MANAGEMENT
 Treatment of DM first requires assessing the potentially affected organs, namely the
skin, muscle, and lungs
 Screening for malignancy is critical because the treatment of a cancer associated
with DM may result in a reduced disease severity
 Monitoring of extracutaneous disease: muscle disease, lung disease and cardiac
disease
 Strength training has been shown to improve muscle strength and function and
aerobic exercise has been shown to improve endurance
INTERVENSI

1. Topical Therapy
• Photoprotection
• Topical glucorticoids→reducing erythema, scale, and pruritus
• Topical calcineurin inhibitors→ tacrolimus 0.1% ointment or pimecrolimus 1% cream
2. Systemic Therapy
• Systemic corticosteroids are first-line therapy in the treatment of
myositis→prednisone monotherapy at doses greater than 0.5 mg/kg/day
• Corticosteroid-sparing agents→Mycophenolate mofetil, at dosages of 2 to 3 g/day
• Antimalarials → first-line agents for skin disease
• Methotrexate
• Calcineurin inhibitors
• Intravenous immunoglobulin (IVIG)