Inverse Gottron’s sign in Anti TIF1 gamma positive juvenile dermatomyositis

Dermatomyositis is a rare idiopathic inflammatory myopathy characterized by distinctive cutaneous findings and proximal muscle weakness. We describe a 15-year-old girl who presented with a six-month history of heliotrope rash and V-sign erythema, accompanied by symmetrical proximal muscle weakness. Further examination revealed Gottron’s papules and the rare manifestation of inverse Gottron’s papules. Laboratory evaluation revealed elevated muscle enzymes. An extended myositis antibody panel demonstrated strong anti-TIF1γ positivity, while anti-MDA5, Mi-2, Jo-1, and other myositis-specific antibodies were negative, confirming a diagnosis of dermatomyositis. Screening excluded interstitial lung disease and underlying malignancy. Inverse Gottron’s papules are usually associated with anti-MDA5 dermatomyositis and rapidly progressive interstitial lung disease; their occurrence in anti-TIF1γ–positive juvenile cases is scarcely documented. Treatment with oral prednisolone and methotrexate led to gradual resolution of cutaneous lesions and muscular symptoms. This case documents the rare coexistence of inverse Gottron’s papules and anti-TIF1γ seropositivity in juvenile dermatomyositis, underscores the diagnostic utility of this atypical palmar cutaneous sign, highlights the clinical phenotype associated with anti-TIF1γ positivity, and emphasizes the importance of continued malignancy surveillance despite the lower cancer risk in pediatric cohorts.