Correspondence Address:
Suresh Kumar Jain
From the Department of Dermatology, Venereology and Leprology, Government Medical College, Kota, Rajasthan
India

Full Text



Sir,

Juvenile dermatomyositis (JDM) is a rare autoimmune multisystem disorder which primarily affects skin and striated muscles. Its annual incidence is two to four cases per million children per year with female prepordance.[1]

A 2-year 8-month-old Indian girl presented to us with complains of difficulty in getting up from the sitting position, since 6 months. She had multiple rashes over face, upper torso and extremities which get aggravated on sun exposure since 4 months. She also had a history of intermittent low-grade fever since 3 months, although never documented.

On examination, she had dusky erythematous macular rash over peri-orbital area (heliotrope rash), upper back and neck (shawl sign) [Figure 1]a, and upper chest (v sign) [Figure 1]b. Symmetrically distributed discrete papules and plaques over extensors of metacarpophalangeal, proximal interphalangeal, distal interphalangeal joints (Gottron papules) [Figure 2]a and [Figure 2]b and elbows [Figure 2]d were noted. Similar lesions were present over lower extremities [Figure 2]c.{Figure 1}{Figure 2}

Nail fold dermoscopy revealed dilated, tortuous capillary loops, capillary haemorrhages and decreased density of capillary loops [Figure 3].{Figure 3}

Neurological examination revealed symmetrical proximal muscle weakness of lower limbs (positive Gower's sign) with 4/5 power at bilateral hip.

On laboratory examination antinuclear antibody was moderately positive (23 UNITS) with elevated lactate dehydrogenase levels, that is, 568 U/L (normal range: 130–240 U/L). Electromyography (EMG) was suggestive of the myopathic pattern.

She was treated with 15 mg/day prednisolone and 5 mg/week methotrexate. On follow-up after 4 weeks she had improvement in muscle power and mild improvement in skin lesions.

JDM is the most common inflammatory myositis in children, classified under idiopathic inflammatory myositis (IIM). It encompasses ~85% of IIM cases, while polymyositis forms <5% of the pediatric IIM cases. Mean age of onset is about 7 years with female preponderance. Characteristic peri-orbital violaceous discoloration (heliotrope rash) as well as gottron papule/sign is almost pathognomic of disease.[2]

Gottron's papules (shiny violaceous papules/plaques) are usually seen over metacarpophalangeal joint, proximal and distal interphalangeal joints, and less commonly over extensor aspects of elbow and knee. No lesions of calcinosis cutis were observed in our case. About 30% of JDM cases present with calcinosis cutis lesions usually after 1–3 years of disease onset and most commonly affected sites include elbow, knee, digits and gluteal region.[3]

In JDM, distinctive nail fold capillary changes include dilated capillary loops, thrombosis and haemorrhage, dropout of vessels, torturosity, bushy-loop formation and arborized clusters of giant capillary loops. Decrease in density of capillaries on capillaroscopy correlates with disease activity.[4]

Till date there are no definitive treatment guidelines for JDM. Standard management is with high dose oral steroid (prednisolone upto 2 mg/kg) until improvement in clinical or laboratory parameters are observed. Early introduction of immunosuppressives such as methotrexate with steroid decrease the incidence of calcinosis, decrease the course of active disease, prevent flares and acts as steroid sparing agent.[5]

To the best of our knowledge, this is the youngest reported case of JDM from India. Any child presenting with regression of gross motor milestones with typical rash must raise suspicion for JDM. As most important determining factor for better outcome in JDM is early diagnosis and administration of high-dose steroid.

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