Kuliah HSP UWK
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PURPURA HENOCH-SCONLEIN
Haryson Tondy Winoto, dr. Msi.Med, Sp.A
Bag. Ilmu Kesehatan Anak
Universitas Wijaya Kusuma Surabaya
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• = Purpura anakfilaktoid
• = Purpura Non-Trombositopenik
• Sindrom klinis akibat vaskulitis pembuluh darah kecil yang ditandai lesi kulit spesifik berupa purpura nontrombositopenik, arthritis atau atralgia, nyeri abdomen atau perdarahan gastrointestinal dan kadang-kadang nefritis atau hematuria
• Umur :
• 2-15 tahun, puncak 4-7 tahun
• Laki > perempuan (1,5:1)
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Etiologi :
• Belum diketahui
• Diduga :
Genetik
Infeksi Traktus respiratorius bag atas ( sp : Haemophillus,Mycoplasma,Parainfluenza,Legionella,Yersinia,Salmonella dan Shigella) , Adenovirus, Varicella
Makanan
Imunisasi (Varisela,rubela,Rubeola,Hepatitis A,HepattisB)
Obat-obatan (ampicillin,Kina,eritromisin)
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Patofisiologi
• Biopsi lesi kulit dan Ginjal : deposit komplek imun yang mengandung Ig A
• Aktivasi komplemen mediator inflamasi inflamasi pembuluh darah kecil kulit, ginjal,sendi, gastrointestinal
• Infiltrasi lekosit PMN di pembuluh darah nekrosis
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Gejala klinis
• Makula eritematosa palpable purpura Pressure-bearing Surface
• Kelainan kulit 100% dijumpai pada pasien yang berobat
• ± gatal
• Angioedema kelopak mata,bibir dan punggung tangan/kaki
• Edema scrotum
• Demam, nyeri kepala dan anoreksia
• Artralgia/arthritis migran sendi besar >>
• Nyeri abdomen/perdarahan GI tract periumbilikal
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• Ginjal : hematuria,proteinuria SN dan Nefritis
• 1 Bulan setelah onset pd kulit
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Pemeriksaan Penunjang
• Trombosit : N atau ↑
• Lekositosis moderat
• Anemia normokrom
• Eosinofilia
• LED ↑
• Ig A darah ↑
• Hematuria,proteinuria, klirens creatinin ↓
• Darah pada feses
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Penatalaksanaan
• Supportif : hidrasi,nutrisi,keseimbangan elektrolit
• Simtomatis : analgesik
• Kelainan Ginjal : kortikosteroid kombinasi dg Imunosupresan
• Kortikosteroid : manifestasi vaskulitis pada SSP,paru,testis ; nyeri abdomen berat,perdarahan GI, edema dan SN persisten
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Complications of Henoch-Schönlein Purpura
• Hepatosplenomegaly
• Myocardial infarction
• Pulmonary hemorrhage
• Pleural effusion
• Unnecessary abdominal surgery
• Intussusception
• Hemorrhage
• Shock
• Gastrointestinal bleeding
• Bowel infarction
• Renal failure
• Hematuria
• Proteinuria
• Seizures
• Mononeuropathies
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Selected Differential Diagnosis of Henoch-Schönlein Purpura
• Acute abdomen • Meningococcal meningitis or septicemia • Rheumatoid arthritis • Rheumatic fever • Idiopathic thrombocytopenic purpura • Systemic lupus erythematosus • Child abuse • Drug reactions • Bacterial endocarditis • Rocky Mountain spotted fever
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Prognosis
• Baik sembuh spontan dalam beberapa hari atau minggu (4 minggu)
• Rekurensi 50 % kasus
• Prognosis buruk bila : penyakit ginjal setelah 3 minggu onset, eksaserbasi yang dikaitkan dengan nefropati,penurunan aktivitas faktor VIII, hipertensi dan gagal ginjal