DERMATOMYOSITIS

A DISEASE THAT INVOLVES THE SKIN, MUSCLES AND BLOOD VESSELS. Dermatomyositis is one of the idiopathic inflammatory
myopathies(IIM). The IIM are a group of genetically determined autoimmune disease that predominantly target the skin and skeletal
muscle. This leads to inflammatory skin lesions and skeletal muscle weakness. The IIM includes mainly DERMATOMYOSITIS,
POLYMYOSITIS AND INCLUSION BODY MYOSITIS.
The Bohan classification of DM-PM:
• Polymyositis
• Dermatomyositis
• PM/DM associated with malignancy
• PM/DM associated with connective tissue disorder
• Childhood dermatomyositis
Diagnostic criteria :
(1) Typical skin rash
(2) Symmetric proximal muscle weakness
(3) Abnormal muscle biopsy
(4) Abnormal EMG
(5) Elevation of skeletal muscle derived enzyme
F:M= 2:1
AGE OF PRESENTATION= 40-60 YEARS (Most common)
Chiidhood DM= usually starts at <10 years of age.
Clinical feature:
(A) Skin: The classic cutaneous manifestations of DM are:
* Pathognomonic skin lesions:
GOTTRON PAPULE: Violaceous papules overlying dorsolateral aspect of IP and MCP joints. When fully formed, they
become slightly depressed in the center assuming white atrophic appearance.
GOTTRON SIGN: Symmetric CMVE with or without edema overlying dorsal aspect of the IP/MCP joints, olecranon
fossa, patella and medial malleoli.
• Characteristic skin lesion:
HELIOTROPE RASH: Periorbital CMVE with or without associated edema of the eyelids and periorbital tissue.
CMVE: over the following areas—
 Dorsal aspect of hand and fingers
 Extensor aspect of arm and forearm
 Deltoid
 Posterior shoulder and neck
 V area
 Thigh
 Central aspect of face, forehead and scalp.

PERIUNGUAL TELANGIECTASIA: With or without dystrophic cuticles and cuticular hemorrhage.

MECHANIC’S HAND LESION
• Other skin lesions:
Poikiloederma atrophicans vasculare: Over posterior shoulder, back, buttock and V area.
Calcinosis cutis
Dermograpism,Urticaria
Bullous lesions
Panniculitis
Livedo reticularis
Erythema nodosum, Erythema multiforme
Follicular keratosis
Diffuse alopecia.
Calcinosis cutis in DM:
In Adults: Calcinosis presents as FIRM DERMAL OR SUBCUTANEOUS PAPULES AND NODULES that are most prominent over the
ELBOWS AND KNEES.
In Children: 4 types of calcinosis occur:
(1) Superficial(DERMAL) pattern consisting of small dermal nodules and plaques
(2) SUBCUTANEOUS nodules
(3) Along FASCIAL PLANES in muscles and tendons
(4) Severe EXOSKELETON pattern of deposition.

(B) Muscle: The most common presentation is symmetric proximal muscle weakness. This can be accompanied by
• involvement of the tongue, pharynx and esophagus leading to nasal speech, dysphagia, diverticular outpouching
of esophagus
• Impaired muscular activity of intestine and colon leading to pneumatosis intestinalis and diverticula of the
colon.
• Weakness of the ocular muscle
• Involvement of the diaphragm and intercostals muscle leading to respiratory difficulty.
Muscle involvement is confirmed by:
• Muscle enzyme elevation: CK-MM(most sensitive) aldolase, AST, ALT, LDH.
 • EMG: Shows a myopathic potential c/b :
 Short duration, low amplitude polyphasic units on voluntary activation
 Increased spontaneous activity with fibrillations
 Complex repetitive discharges
 Positive sharp waves.
• Muscle imaging: MRI
• Muscle biopsy: shows
 Muscle fibre degeneration and regeneration
 Perifascicular atrophy( in PM there is endomysial inflammation, prominent CD8
lymphocytes): DIAGNOSTIC of DM.
 Capillary injury(in PM there is no microangiopathy)
 Perivascular infiltrate of T lymphocytes (mainly CD4) and histiocytes that extends onto
the necrotic muscle but never the nonnecrotic muscle

© Cardiovascular system: Myocarditis, cardiomyopathy, conduction defects and cor pulmonale.

(D)Respiratory system: Interstitial pneumonitis, respiratory muscle weakness leads to nonproductive cough, dyspnoea, hypoxemia
and radiological infiltrates. This leads to pulmonary hypertension and ultimately to cor pulmonale.
(E) Retinitis: It produces fluffy exudates around the papilla and along veins.
(F) Kidney: Myoglobulinuric ARF may be produced.
(G) Arthritis
(H) Raynauds phenomenon.
ETIOPATHOGENESIS:
A. Autoimmune: Evidences in support are:
 Association with other autoimmune disease
 Presence of various autoantibodies
 A/W Histocompatibility genes (HLA-B8 in childhood DM; HLADR3 and HLAB14 in adult DM.)
 Evidence of T cell mediated myotoxicity or complement mediated damage in microangiopathy.
ANA is positive in 60-80 % cases.
Anticytoplasmic antibodies are divided into:
 Myositis specificAb: Jo-1, PL7, PL12, OJ, Mi-2, SRP.
 Myositis associated: PM/Scl, Ro/SSB, and U1 RNP.
Antisynthetase syndrome: Antisynthetases target the various aminoacyl t-RNA synthetases. Of these Ab to Jo-1( histidyl t-RNA
synthetase) is most common. It is associated with:
 Fever
 Interstitial lung disease
 Raynauds
 Polyarthritis
 Mechanic’s hand
 Incomplete response to therapy
 A/W HLA-DR3, DRw52.
 Less frequently display classic skin changes of DM.

(B) Virus: coxsakie

Influenza
Paramyxovirus
Mumps
CMV
EBV
HIV
HTLV-1
© Other environmental factors:
 Silicon breast implant
 Bovine collagen implant
 Drugs: pennicilamine, statins, tryptophan, hydroxyurea, phenytoin
IMMUNOPATHOLOGIC MECHANISM:
In DM,
 The infiltrates have higher number of B cells, higher ratio of CD4 cells, proximity of CD4 cells to B
cells and macrophages and a relative absence of lymphocyte invasion of nonnecrotic muscle fiber:
all these suggest a humoral immune response.
 The antibodies possibly target the microvascular antigen and this triggers the
complement activation. The end result is necrosis of endothelial cells and muscle fibre
destruction.
 This results in perifascicular atrophy(C/B 2-10 layers of atrophic fibres at the periphery
of fascicles).
 Complement activation also causes release of proinflammatory cytokines: inflammatory cell infiltrate
in the necrotic muscle( not in healthy muscle). In DM, there is interfascicular inflammation
In PM,
 There is no evidence of microangiopathy and muscle ischaemia.
 However, there is presence of CD8+ cytotoxic T cells along with macrophages invading and
destroying healthy nonnecrotic muscle.
 In PM, there is endomyseal inflammation.
 These suggest an ADCC mediated by CD8+ cytotoxic T cells.
SPECIAL CASES:
(A) Dermatomyositis in childhood:
• Clinically resembles the adult form
• Course is much more severe.
• Widespread vasculitis of small arteries, capillaries and veins of skin, muscle, subcutaneous tissue and GIT.
• More calcinosis and contractures .
• May take many years for the disease to burn out.
• Recurrence rare
• More psychological difficulties.
Dermatomyositis in pregnancy:
 Fertility is decreased.
 Pregnancy mostly increases and precipitates DM.
 Overall fetal loss is >50%. Half of the pregnancies end prematurely. Pregnancy should be
planned at the time of remission and patient monitored every month.
Associations of DM:
(1) Malignancy :
 It is more A/w DM than PM.
 Malignancy not associated with childhood DM.
 Most frequent tumors are ovarian ca, breast ca, melanoma and colon ca. Others include: lung ca, GI ca,
renal ca, testicular ca, nasopharyngeal ca, and lymphoma.
(2) Other CTD: SLE, RA, PSS, MCTD, Sjogren( DM+ PSS : most common overlap).
(3) Other AI disease: Myasthenia gravis, thymoma, Hashomoto thyroiditis, Behcet’s disease, Crohn’s disease, Primary
biliary cirrhosis.
(4) Complication of drugs: D-pennicilamine, contaminated Tryptophan.
Diagnosis of DM:
• CLINICAL FEATURES: Typical rash with proximal muscle weakness
• Muscle biopsy, EMG, Raised CPK and SGOT, LDH.
• Radiology to show Calcinosis in late stages. This distinguishes it from SLE. Again in PSS, calcium deposits
are found only in hands and around elbows and knees. But in DM , the calcinosis is widespread.
• Anticytoplasmic antibodies:

autoAb % Target antigen Clinical association

115kB 80 Uncharacterized polypep. Clinically amyopathic DM

Jo-1 20 Histidyl t RNA synthetase PM, antisynthetase syndrome
Mi-2 15 Helicase nuclear protein Skin manifestation of DM
SRP 5 Signal recognition particle Fulminant DM/PM, cardiac involvement
PL-7 3 Threonyl t RNA synthase Antisynthetase syndrome
PL-12 3 Alanyl t RNA synthetase Antisynthetase syndrome
ANA 40 Clinically amyopathic DM
SsDNA 40 Overlap with SLE, Sscl.
PM/Scl 40 Overlap with Sscl
Ku 3 DNA repair protein Overlap with Sscl
U2RNP 1 Overlap with Sscl
Ro 15 RNP Overlap with SSJ,SLE, neonatal LE, SCLE.
U1RNP 10 U1RNP Overlap with CTD.
Differential diagnosis:
(A) Systemic sclerosis: difficult to distinguish from DM esp. when systemic sclerosis is in healing stage and there
is heliotrope rash with periungual telangiectatia
• Muscle changes identical in both, visceral changes more in PSS.
• ANA common in serum of PSS; infrequent in DM.
• Anticytoplasmic antibodies in DM.
• Gottron pap and Gottron sign.
(B) SLE:
• Rash is violaceous in DM but red/pink in SLE.
• Distribution of rash :
DM SLE
Eyelid, periorbital malar eminence
Scalp Scalp
Periungual telangiectasia Oral mucosa
Knuckles Dorsal fingers
• Alopecia more in SLE
• Gottron papule
• Pruritus more in DM
• DEJ immune deposits more in SLE
• Dermal microvessel C5-9 deposits more in DM.
• Anti ds-DNA , anti-Sm more in SLE.
• Anticytoplasmic antibodies in DM.
 • Malignancy association in DM.
(C) Causes of Proximal muscle weakness:
 Infectious myopathies: HIV, HTLV-1, EBV, HBV, Coxsakie
 Drug induced: D-penicillamine, zidovudine, alcohol, steroid, isotretinoin, statin, colchicines.
 Endocrine disorders: Cushing, hypothyroidism, hyperthyroidism, diabetic neuropathy,
hypokalemia.
 Neurologic: Myasthenia gravis, GB syndrome.
 Other myopathies: muscular dystrophies.

PROGNOSIS:
 Variable
 It is better in DM c.f. PM
 It is better with calcinosis
 It is better if high dose of steroid is started early. Combined therapy with steroid and azoran
yield better result.
After an acute phase lasting for some months, most settle slowly and burn out in time usually over some years.
TREATMENT:
(1) LEVEL 1: STEROID
Dose: 1-1.5 mg/kg/day for 1 month-----then slowly taper over 10 weeks to 1 mg/kg on alternate days----- then taper by
5-10 mg monthly till dose is titrated.
(2) LEVEL 2: IMMUNOSUPPRESIVES
 AZORAN: upto 3 mg/kg/day
 METHOTREXATE: Starting from 7.5 mg and gradually increase to 25 mg weekly.
 Cyclophosphamide
 Chlorambucil
 Cyclosporine
 Mycophenolate mophetil.
(3) IMMUNOMODULATOR:
Intravenous gamma globulin
(4) TRIAL:
Anti- TNF alpha (etanercept/ infliximab)
(5) Treatment for dystrophic calcification: Warfarin(1mg daily), potassium PABA(15-25mg daily), EDTA, Bisphosphonate,
Colchicine.
(6) Treatment for cutaneous manifestations:
HCQS, Dapsone, Steroid