Pre-exam 1.Inspect 2.Palpate 3.Gait+Back 4.ROM 5.GPE 6.Dev Description Differentials Invest MDT Prognosis Tips paeds.online

🧬 Juvenile Dermatomyositis (JDM) CPSP Short Case

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Pre‑exam protocol (WIPER) · Locomotor

Wash hands (sterilizing solution) Warm hands Introduce & rapport Position: standing / sitting / lying Exposure (shirt off, trousers rolled) Approach from right side
“Kissi jorr main dard tuu nahi?” “Bachaa Chal saktaa hai?” “Muh kholo, ungli rakho” “Pair se khara ho jao” “Zara uth kar dekho”
STEP 1 Inspection (Skin & Joints)
  • Heliotrope rash (violaceous periorbital, esp. upper lid) – 66-83%
  • Gottron's papules (PIP, DIP, elbows, knees) – pink/pale shiny plaques
  • Shawl sign (erythema over chest/back, sun-exposed V area)
  • Mechanic's hands (thickened scaly palms/flexor tendons) – rare, anti-Jo-1
  • Cutaneous ulcers, calcinosis (long-standing), periungual telangiectasia
  • Muscle wasting, joint swelling/deformity, lipodystrophy
STEP 2 Palpation (Muscle & Joints)
  • Muscle tenderness (50% cases) – proximal muscle groups
  • Joint warmth, effusion, synovitis (52-58% have arthritis/arthralgia)
  • Temperature (compare bilaterally)
  • Subcutaneous nodules, calcinosis (firm nodules/plaques)
  • Pulses, BP, lymph nodes
STEP 3 Gait & Back (Proximal weakness)
  • Ask: “Bachaa Chal saktaa hai?” – waddling / lordotic gait, toe walking
  • Gower's sign (climbing up legs to stand) – proximal weakness
  • Back: spinal tenderness, deformity, scoliosis
  • Squat (proximal weakness), stand on one leg (Trendelenburg)
  • Observe child rolling to side to sit up (truncal weakness)
STEP 4 Range of Motion

Upper limb

  • Fingers: make fist, prayer sign, reverse prayer
  • Elbow: flexion, extension, supination, pronation
  • Shoulder: abduction, adduction, rotation, circumduction

Lower limb & spine

  • Hip: flexion, extension, abduction, adduction, rotation
  • Knee: flexion, extension, internal/external rotation
  • Ankle/foot: dorsi/plantar flexion, inversion, eversion
  • TMJ: open mouth (3 fingers), neck movements

Always stabilise pelvis / shoulder girdle; do passive movements only in affected joints.

STEP 5 Relevant GPE (multi-system)
  • Eyes: slit lamp (uveitis), periungual telangiectasia, nailfold capillaries
  • CVS: pericarditis, myocarditis, conduction defects (ECG)
  • Chest: respiratory effort, signs of aspiration, interstitial lung disease
  • Oral: palatal weakness, dysphonia, dysphagia, nasal speech
  • Abdomen: hepatosplenomegaly, GI vasculitis (pain, bleeding)
  • Hands: Gottron's, mechanic's hands, calcinosis, lipodystrophy
  • Genitalia: Tanner staging, contraception counselling (teratogenic drugs)
STEP 6 Functional & Dev Assessment
  • Comb hair, dress/undress, write name (fine motor)
  • School performance, mobility, activities of daily living
  • Measurements: true leg length (ASIS to medial malleolus), apparent (umbilicus to malleolus)
  • Anthropometry: height, weight, BMI (growth failure, steroid effects)
  • CNS: mood, cognitive function, depression screening
Description template
“Thank you sir. I would like to complete my examination by doing [missed steps: vitals / anthropometry / slit lamp]. This is a [age]-year-old child, conscious, cooperative. No acute distress. Skin: [heliotrope rash / Gottron's papules / shawl sign / mechanic's hands / calcinosis]. Gait: [waddling / lordotic], Gower sign [positive/negative]. Motor: proximal weakness (power [4/5]), distal [5/5]. Muscle tenderness [present/absent]. Joints: [number] swollen joints, ROM reduced at [joints]. No fixed flexion deformity. GPE: CVS [normal / pericardial rub], chest clear, no visceromegaly, no lymphadenopathy. He can dress/undress himself and is able/not able to walk. True leg length: [R] cm, [L] cm. Apparent leg length: [R] cm, [L] cm. I would like to do a slit lamp exam, contrast swallow, and muscle enzymes.”
Differentials

Infections / Post-infectious

  • Influenza A/B, Coxsackievirus B
  • Parvovirus B19, EBV
  • Group A streptococcus

Muscular dystrophies

  • Duchenne / Becker MD
  • Limb-girdle MD

Autoimmune / Rheumatologic

  • SLE (no nasolabial sparing)
  • Polymyositis
  • Mixed connective tissue disease
  • Juvenile idiopathic arthritis

Other

  • Drug-induced myositis
  • Graft-versus-host disease
  • Myasthenia gravis
Investigations
DIAGNOSISClinical diagnosis (characteristic rash + proximal weakness). Muscle biopsy (definitive). MRI (T2 hyperintensity). EMG (myopathic).
SUPPORTIVECK, AST, aldolase (elevated). ESR (normal or elevated). ANA+ (80%). Myositis-specific antibodies (anti-Jo-1, anti-Mi-2, anti-p155/140). Negative RF, anti-SSA/SSB/Sm.
EXCLUDE OTHERSMuscle dystrophy gene testing, thyroid function, infectious workup (viral, bacterial), drug screen
RULE OUT COMPLICATIONSContrast swallow (aspiration), PFTs (restrictive), ECG/ECHO (myocarditis), slit lamp (uveitis), X-ray (calcinosis), bone profile

Nailfold capillaroscopy – dropout of capillary loops (80%).

MDT Management
Pharmacological
Corticosteroids (IV methylprednisolone 30 mg/kg/day ×3, then oral prednisone 2 mg/kg/day). Methotrexate (steroid-sparing). Hydroxychloroquine (skin). Rituximab (refractory). IVIG, Mycophenolate, Cyclosporine, Cyclophosphamide.
Supportive
Nasogastric/gastrostomy feeds (aspiration). Ventilatory support (respiratory weakness). Sunscreen (SPF 50+ daily). Calcium & Vitamin D (steroid osteoporosis).
PT & OT
Aggressive physical therapy (ROM, strengthening). Occupational therapy (ADLs). Bed rest NOT indicated – weight bearing prevents contractures.
Cardiology / Pulmonology
ECG, ECHO (myocarditis, conduction defects). PFTs (restrictive lung disease). Tracheostomy if severe respiratory weakness.
Psychology & Counselling
Mental health support, school liaison, family counselling. Avoid live vaccines (methotrexate).
Nutrition
Vitamin D + calcium supplementation. Full bowel rest if GI vasculitis. Monitor growth, bone health (DEXA).
Prognosis & Counseling
Mortality rate~1%
Active symptoms persist<1.5 years
At 7-year follow-up75% little/no residual disability; 25% chronic weakness; 40% chronic rash
Calcinosis riskRelated to severity and delay in therapy; affects ~40%
Long-term medicationUp to one-third need long-term therapy

Support: Myositis Association · Arthritis Foundation (www.arthritis.org)

Tips & Tricks

Time taking exam – practice systematically; offer missed steps at the end.

Never inflict pain – examine tender joints last, look at child’s face.

Expose properly – check for Gottron's, heliotrope, shawl sign, calcinosis.

Nailfold capillaroscopy – use immersion oil + ophthalmoscope at 40+ diopters.

Don't forget cardiac – myocarditis, pericarditis, conduction defects.

Dysphagia/aspiration – contrast swallow; nasal speech, palatal weakness.

Calcinosis – X-ray; early therapy reduces incidence.

Nelson Textbook of Pediatrics · CPSP short case · Juvenile Dermatomyositis (JDM)