JUVENILE DERMATOMIYOSITIS: PENEGAKAN DIAGNOSIS

Flora Ramona Sigit Prakoeswa, . Suswardana, Retno Danarti

DOI: https://doi.org/10.23917/biomedika.v6i1.285

Abstract

Juvenile dermatomiyositis (JDM) merupakan suatu penyakit autoimun infl amatif yang jarang dijumpai dan secara khas ditandai oleh adanya lesi-lesi kulit tipikal serta kelemahan otot proksimal yang simetris. Etiologi penyakit tersebut belum diketahui dengan pasti, namun demikian patogenesisnya diketahui bersifat multifaktorial meliputi faktor genetik, paparan sinar ultra violet (UV) serta infeksi oleh berbagai mikroba seperti virus Coxsackie atau Borrelia burgdorferi. Penegakan diagnosis JDM adalah berdasarkan kriteria Bohan-Peter meliputi: 1) kelemahan otot proksimal simetris, 2) peningkatan enzim otot, 3) infl amasi miopati pada hasil biopsi otot 4) gambaran miyopati pada hasil pemeriksaan EMG, dan 5) lesi kulit tipikal. Ditemukannya papul-papul Gottron dan heliotrope rash di kulit, serta adanya kelemahan otot proksimal yang simetris, merupakan manifestasi klinis yang khas untuk JDM. Terapi lini pertama penatalaksanaan JDM adalah kortikosteroid sistemik. Dilaporkan satu kasus JDM pada seorang anak perempuan berusia 6 tahun. Diagnosis ditegakkan berdasarkan adanya lesi kulit tipikal, kelemahan otot proksimal simetris dan peningkatan kadar enzim penanda infl amasi jaringan muskuloskeletal. Pasien berespon baik terhadap pemberian kortikosteroid adekuat serta tabir surya.


Kata Kunci: Juvenile dermatomiyositis, systemic corticosteroid

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