SCIENTIFIC
Classic Presentation of Juvenile Dermatomyositis in a Three-Year-Old
AUTHORS : Sabah Sarwari , MD 1
Nicholas Mains , DO 1 Evan Abbey , MD 2
1
West Virginia University Medicine Children ’ s Hospital
2
West Virginia University School of Medicine
CASE PRESENTATION
A 3-year-old boy with a past medical history of eczema and asthma presented with a two-month history of progressively worsening proximal lower extremity muscle weakness and pain with a new rash on his chest and face . His mother reported that he was no longer able to sit on the floor with his legs extended , climb up stairs , get out of his cot at school unassisted , or jump with his feet leaving the floor . She described an abnormal gait with out-toeing of his feet bilaterally as well as pain with any weight bearing on his lower extremities . She had also noticed a new rash around his eyes and chest that was different from his typical eczema . Birth history was unremarkable , and his paternal grandfather had systemic lupus erythematosus ( SLE ).
On physical examination , he was afebrile ( 36.9 ° C ) with a heart rate of 116 , blood pressure of 103 / 64 , respiratory rate of 20 , and oxygen saturations of 95 % to 100 %. His weight was in the 86th percentile ( 17.2kg ) and height in the 40th percentile ( 97.1cm ). He appeared in no acute distress with normal cardiovascular , pulmonary , and abdominal examinations . No lymphadenopathy was appreciated , and he had normal active range of motion of all his joints without any edema or erythema . He had erythematous , scaly , and pruritic plaques on the flexor surfaces of his elbows and knees bilaterally ; erythematous papules over his metacarpophalangeal and proximal interphalangeal
ABSTRACT
A 3-year-old patient presented with classic symptoms of a positive Gower sign , Gottron papules , and laboratory and imaging findings consistent with juvenile dermatomyositis
joints ( Figure 1 ); hypopigmented patches on his forehead , chest , and back ; and violaceous patches around his eyes ( Figure 2 ) that became more prominent when crying . Cranial nerves II – XII were intact without any fasciculations or atrophy of his tongue noted . Muscle bulk was normal , but he had difficulty with arm raises and displayed a positive Gower sign . All reflexes were 2 + and symmetric bilaterally , and there was no dysmetria or ataxia noted . However , he walked with his hips in external rotation with subsequent out-toeing of his feet bilaterally and cried until he was picked up by his mother and was no longer bearing weight on his lower extremities .
A complete blood count with a differential , complete metabolic panel , C-reactive protein , erythrocyte sedimentation rate ( ESR ), creatinine kinase ( CK ), aldolase , lactate dehydrogenase ( LDH ), vitamin B12 , vitamin D , folate , and thyroid stimulating hormone were obtained and found to be remarkable for an elevated ESR ( 28 mm / h ; 0 – 15 mm / h ), CK ( 703 U / L , 11.74μk / L ; 30 – 175 U / L , 0.50 – 2.92 μk / L ), aldolase ( 14.5 U / L , 0.24 μk / L ; < 14.5 U / L , < 0.24 μk / L ), and LDH ( 498 U / L , 8.32 μk / L ; 175 – 320 U / L , 2.92 – 5.34 μk / L ). A sedated magnetic resonance image ( MRI ) of the cervical spine was obtained and showed diffuse edema and enhancement of the muscles consistent with myositis ( Figure 3 ). The patient was determined to have juvenile dermatomyositis ( JDM ) based on his clinical presentation and the above laboratory values . He was initially treated with high dose intravenous methylprednisolone for the first 72 hours and transitioned to high dose oral prednisolone with concurrent use of subcutaneous methotrexate and
( JDM ). Our case adds to the scant published reports of JDM in younger children , who may not necessarily present in this classic manner .
FIGURE 1
Erythematous papules overlying the metacarpophalangeal ( arrows ) and proximal interphalangeal ( arrowhead ) joints .
FIGURE 2
Periorbital violaceous patches ( arrows ).
FIGURE 3
Diffuse edema and enhancement of the muscles seen on the Magnetic Resonance Imaging ( MRI ) of the Cervical spine .
West Virginia Medical Journal • September 2024 • 21