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Year : 2017  |  Volume : 18  |  Issue : 2  |  Page : 107-109

A rare case of juvenile dermatomyositis and review of literature

Department of Pediatrics, Index Medical College Hospital and Research Center, Indore, Madhya Pradesh, India

Date of Web Publication27-Mar-2017

Correspondence Address:
Anjali T Bharani
Department of Pediatrics, Index Medical College Hospital and Research Center, Indore - 452 001, Madhya Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2319-7250.202994

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Idiopathic inflammatory myopathies are rare group of systemic connective tissue diseases. The hallmark of these disorders is symmetrical chronic inflammation and weakness of proximal muscles. Juvenile dermatomyositis (JDM) is the most common inflammatory myositis in children. We describe a rare case of JDM in a 4-year-old female child who presented with characteristic cutaneous rash and proximal muscle weakness.

Keywords: Gottron papules, heliotrope rash, juvenile dermatomyositis

How to cite this article:
Bharani AT, Harshey M, Khurana R, Raipurkar S. A rare case of juvenile dermatomyositis and review of literature. Indian J Paediatr Dermatol 2017;18:107-9

How to cite this URL:
Bharani AT, Harshey M, Khurana R, Raipurkar S. A rare case of juvenile dermatomyositis and review of literature. Indian J Paediatr Dermatol [serial online] 2017 [cited 2020 Nov 25];18:107-9. Available from: https://www.ijpd.in/text.asp?2017/18/2/107/202994

  Introduction Top

Juvenile dermatomyositis (JDM) is a rare autoinflammatory myositis. It has an incidence of 1.9–4 per million children per year and prevalence of 2.5/100,000.[1] It is characterized by its typical cutaneous findings and focal areas of myositis resulting in progressive proximal muscle weakness. Genetic factors and environmental triggers such as enterovirus and group B streptococcus infection and ultraviolet radiation exposure are likely to play an important role in the etiology.[2] JDM has been associated with significant mortality and morbidity in developing countries.[3],[4]

  Case Report Top

A 4-year-old female child presented to us with a history of gradually progressive weakness in the lower limbs and itchy rash over the face and body for 6 months. She also complained of fever for 1 month and pain in the left elbow joint for 15 days.

On examination, she had erythematous rash around the eyes and cheeks (heliotrope rash) [Figure 1] and a nonhealing ulcer in the right axilla. Erythematous patches were noted on the metacarpophalangeal and interphalangeal joints of the dorsum of both hands (Gottron's papules) [Figure 2]. Her left elbow was swollen with restricted range of movement. She had symmetrical proximal muscle weakness in both the lower limbs with hypotonia. Power at hip joint was 2/5 and at knee 3/5, and ankle joint 4/5 on both sides. All deep tendon jerks were nonelicitable. Fasciculations and pseudohypertrophy of muscles were absent.
Figure 1: Erythematous rash around eyes and cheeks (heliotrope rash)

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Figure 2: Erythematous patches on the metacarpophalangeal and interphalangeal joints of hands (Gottron's papules)

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Laboratory investigations revealed normal creatine phosphokinase (CPK): 145.5 U/L (25–200 U/L), serum glutamic pyruvic transaminase/serum glutamic oxaloacetic transaminase: 28/65 U/L (<40 U/L), and C-reactive protein (CRP) and antinuclear antibody (ANA): negative; electromyography (EMG)-nerve conduction velocity study showed small polyphasic motor units with early recruitment suggestive of myopathy. Magnetic resonance imaging (MRI) of the thigh was inconclusive. Muscle biopsy was done which showed patchy mononuclear infiltrate in the interfascicular perimysial septa with degenerated muscle fascicles predominantly in the periphery suggestive of JDM [Figure 3].
Figure 3: Muscle biopsy showing mononuclear infiltrate in the interfascicular perimysial septa with degenerated muscle fascicles in the periphery (H and E, ×40)

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Treatment with oral prednisolone (2 mg/kg/day), methotrexate (0.5 mg/kg/day), and folic acid was started.

On follow-up after 15 days, power at hip joint had improved to 4/5 and rash had subsided.

  Discussion Top

Idiopathic inflammatory myopathies are rare group of systemic connective tissue diseases. The hallmark of these disorders is symmetrical chronic inflammation and weakness of proximal muscles. JDM is the most common inflammatory myositis in children. The peak age of onset of JDM reported is 7 years with female predominance. It is characterized by proximal muscle weakness and distinctive cutaneous findings, but presenting features may be variable and insidious in onset which makes the early diagnosis difficult.[5],[6],[7] Weakness is progressive and can become profound, starting with difficulty in climbing stairs or combing hair, progressing to an inability to roll over in bed. They may develop weakness of neck flexors and abdominal muscles. Furthermore, weakness of palatal/cricopharyngeal muscles may result in nasal voice, swallowing difficulties, and tracheal aspiration.

Characteristic cutaneous findings are reported in 72.9–97% of cases at presentation.[5],[7] The most common of the cutaneous features are heliotrope discoloration, Gottron's papules/sign, and nail fold capillary changes. Calcinosis is reported in 27.7% in a recent Indian study.[3] Calcifications are highly associated with the delayed diagnosis and duration of untreated disease.

Fever, weight loss, fatigue, myalgias, arthritis, lymphadenopathy, and abdominal pain are constitutional symptoms known to be associated with JDM.[5],[7]

Gastrointestinal system involvement may lead to gut vasculitis and perforation; similarly, dyspnea may occur due to respiratory involvement in the form of interstitial lung disease, aspiration pneumonia, or respiratory muscle involvement. Major organ involvement, severe weakness, and ulcerative skin disease put a patient at high risk, and such patient needs urgent attention and treatment under specialist supervision.

Characteristic rash facilitates early diagnosis but should be differentiated from other connective tissue diseases such as systemic lupus erythematosus and scleroderma.

Diagnosis of definite JDM (using Bohan and Peter criteria) requires a characteristic rash (heliotrope and Gottron's papules) plus three of the four muscle features: Symmetrical muscle weakness, muscle biopsy evidence of myositis, elevation of serum levels of muscle associated enzymes, and EMG triad of myopathy.[8] Measurement of muscle-derived enzymes should include CPK, lactate dehydrogenase, alanine aminotransferase, and aspartate aminotransferase.

ANA is positive in approximately 70% of patients but is nonspecific and not diagnostic. Inflammatory markers (erythrocyte sedimentation rate and CRP) may/may not be abnormal despite active disease.

The diagnosis of JDM needs strong clinical suspicion. Even negative initial investigations do not rule out JDM as a diagnosis. In many cases, muscle enzymes may be normal. Indeed in a review of 175 children with JDM, no single test was consistently abnormal.[5] An MRI scoring system has been developed to objectively define acute inflammatory change.[9] MRI using T2-weighted images and fat suppression localizes the active site of disease for diagnostic muscle biopsy and EMG and represents a noninvasive method to estimate inflammation. In fact, MRI study has now replaced the need to do invasive test of muscle biopsy which had been the gold standard.

For typical cases of JDM, prompt institution of immunosuppressive therapy with high-dose corticosteroids (oral or intravenous) and methotrexate has become the standard treatment regimen.[5],[6] This has been shown to shorten the time to remission compared to prednisolone alone, with a good safety profile.[7] Other agents such as intravenous immunoglobulin, cyclosporine, or mycophenolate mofetil may be used in severe cases or in those showing inadequate response to conventional treatment. Topical tacrolimus (0.1%) or topical corticosteroids may help in localized skin disease. Calcium and Vitamin D supplements are also given to correct the decrease in bone mineral density. Steroid should be gradually tapered as a patient shows clinical improvement.

Outcomes have improved with advances in medical treatment. The mortality rate has decreased to <2%, but 51–73% of JDM have been reported to have detectable disease activity after median 16.8 years of follow-up.[10] Delayed or inadequate steroid dose is one of the most important factors associated with poor prognosis and increased the risk of calcinosis. The disease remains a serious illness and carries significant mortality in developing countries. Early diagnosis and aggressive treatment is the key to improving outlook.

Declaration of Patient Consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of Interest

There are no conflicts of interest.

  References Top

Meyer A, Meyer N, Schaeffer M, Gottenberg JE, Geny B, Sibilia J. Incidence and prevalence of inflammatory myopathies: A systematic review. Rheumatology (Oxford) 2015;54:50-63.  Back to cited text no. 1
Ernste FC, Reed AM. Recent advances in juvenile idiopathic inflammatory myopathies. Curr Opin Rheumatol 2014;26:671-8.  Back to cited text no. 2
Prasad S, Misra R, Agarwal V, Lawrence A, Aggarwal A. Juvenile dermatomyositis at a tertiary care hospital: Is there any change in the last decade? Int J Rheum Dis 2013;16:556-60.  Back to cited text no. 3
Singh S, Suri D, Aulakh R, Gupta A, Rawat A, Kumar RM. Mortality in children with juvenile dermatomyositis: Two decades of experience from a single tertiary care centre in North India. Clin Rheumatol 2014;33:1675-9.  Back to cited text no. 4
McCann LJ, Juggins AD, Maillard SM, Wedderburn LR, Davidson JE, Murray KJ, et al. The Juvenile Dermatomyositis National Registry and Repository (UK and Ireland)- clinical characteristics of children recruited within the first 5 y. Rheumatology (Oxford). 2006;45:1255-60.  Back to cited text no. 5
Robinson AB, Hoeltzel MF, Wahezi DM, Becker ML, Kessler EA, Schmeling H, et al. Clinical characteristics of children with juvenile dermatomyositis: The childhood arthritis and rheumatology research alliance registry. Arthritis Care Res (Hoboken) 2014;66:404-10.  Back to cited text no. 6
Ramanan AV, Feldman BM. Clinical features and outcomes of juvenile dermatomyositis and other childhood onset myositis syndromes. Rheum Dis Clin North Am 2002;28:833-57.  Back to cited text no. 7
Bohan A, Peter JB. Polymyositis and dermatomyositis (second of two parts). N Engl J Med 1975;292:403-7.  Back to cited text no. 8
Davis WR, Halls JE, Offiah AC, Pilkington C, Owens CM, Rosendahl K. Assessment of active inflammation in juvenile dermatomyositis: A novel magnetic resonance imaging-based scoring system. Rheumatology (Oxford) 2011;50:2237-44.  Back to cited text no. 9
Sanner H, Sjaastad I, Flatø B. Disease activity and prognostic factors in juvenile dermatomyositis: A long-term follow-up study applying the paediatric rheumatology international trials organization criteria for inactive disease and the myositis disease activity assessment tool. Rheumatology (Oxford) 2014;53:1578-85.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3]

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