dermatosis vesikobulosa
DESCRIPTION
pptTRANSCRIPT
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DERMATOSIS VESIKOBULOSA
Dr.WIDYO ATMOKO,SpKK
Dep. Kulit & Kelamin RSPAD Gatot Soebroto Jakarta
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DERMATOSIS VESIKOBULOSA
• Kronis
• Vesikel & Bula
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Kelompok Dermatosis vesikobulosa
• Pemfigus• Dermatitis herpetiformis• Pemfigoid bulosa• Herpes gestasiones• Chronic Bullous Diseases of Childhood• Pemfigoid sikatrisial• Subkorneal Pustulosa Dermatosis• Epidermolisis bulosa• Subcorneal pustular dermatosis• Porphyria cutanea tarda (PCT)
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PEMFIGUS
• Kumpulan penyakit berbula, kronik
• Bula kendor, intraepidermal →Nikolsky sign (+), akantolisis (+), IgG antigen interseluler di epidermis →serum, terikat pada epidermis.
• Dapat fatal
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4 Bentuk Pemfigus
• Pemfigus vulgaris
• Pemfigus eritematosus
• Pemfigus foliaseous
• Pemfigus vegetan
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PEMFIGUS VULGARIS
♦ Insidens: ▫ 80% dr kasus pemfigus ▫ Pria : Wanita = 1:1 → 40-50 th.♦ Etiologi : ▫ Autoimun♦ Ptogsis : ▫ Otoantibodi (IgG) antigen permukaan sel keratinosit.
▫ Akantolisis (hilangnya adhesi sel epidermis)♦ G klnis : ▫ Mukosa mulut →sariawan tak sembuh-2 ▫ Bula kendor → Nikolsky sign (+)♦ PA : ▫ Bula intra epidermal (suprabasal)♦ Terapi : ▫ Topikal → Kortikosteroid Cyclosporin ▫ Sistemik →Kortikosteroid, Anti inflamatory agent, Immunosupresion agent.
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Terapi sistemik pemfigus
♦ Dosis awal prednison 40-60 mg/hrPrednison 60-150 mg/hr.
♦ Steroid sparing agent:Dapson 100mg/hr, Azathioprine 100-150
mg/hr, Cyclophosphamide 50-150 mg/hr, MTX 15-30 mg/minggu, Gold 50mg/mgg, Mycophenolate mofetil 2 g/hr.♦ Alternatif terapi: Plasmaferesis. IVIG (intravenous ImmunoglobulinG).
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PEMFIGUS ERITEMATOUS (PE)
=Sindroma Sinear-Usher=
G klns: ■ Eritema, skuama, krusta, bula
kendor →Nikolsky sign (+).
■ Leher badan atas, wajah ~ Lupus
Eritematosus.
PA : ■ Bula subkorneal
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PEMFIGUS FOLIACEUS (PF)
G klns: ♦ Eritema, skuama, krusta, bula
kendor →Nikolsky sign (+)
♦ Lesi terbatas pada kulit saja
PA : ♦ Bula subkorneal
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PEMFIGUS VEGETAN
Klasifikasi 2 tipe:1. Tipe Neuman 2. Tipe Hallopeau (Pyodermite vegetante)
Tipe Neuman: Gk/ : Bula kendor → vegetatif & proliferatif & papilomatosa
Daerah intertriginosa, oral hampir terkena
PA: Akantolisis suprabasal (awal) Proliferasi papila -2 kearah epidermis Abses -2 intraepidermal eosinofil (+)
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PEMFIGUS VEGETAN
• Tipe Halopeau:Gk/ : Primer → Pustul- 2 bersatu → vegetatif
Daerah aksila, perineum Oral → granulomatosis seperti beledu
PA: Akantolisis suprabasal (awal) Hiperplasi epidermis dengan abses eosinofilik,
papilomatosis, hiperkeratosis tanpa abses.
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PEMFIGOID BULOSA
• Penyakit kronik • Bula dinding tegang disertai eritem
E/ : Autoimun
Gk/: Bula dinding tegang → Nikolsky sign (-)
Lipatan, fleksor
Mulut → 20% kasus
PA: Bula subepidermal
IF: IgG & C3 tersusun seperti pita di BMZ
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DERMATITIS HERPETIFORMIS
(MURBUS DUHRING) – Penyakit menahun & residif – Polimorfik, berkelompok, simetris – Sangat gatal E/: Tidak diket Patogenesis : Mungkin sebagai antigen gluten
Gk: Anak-2, dewasa Vesikel berkelompok, simetris Enterophatia sensitif terhadap gluten Sangat gatal Vesikel/bula tegang arsiner sirsiner PA: Bula subepidermal IF : IgA pada papila dermal berbentuk granulasi Terapi: DDS 100-300 mg/hari Makanan bebas gluten
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CHRONIC BULLOUS DISEASE OF CHILDHOOD (CBDC)
Dermatosis Linear IgA pada anak
• Dermatosis pada anak • Bula & IgA linear
Gk/:- Vesikel, bula tegang tersusun seperti untaian mutiara. -Mukosa: Mulut & konjuctiva. -Badan, Extremitas. PA: -Bula subepidermal -Vacualisasi sel basal pada ujung papila dermis IF: IgA linier pada BMZTerapi: DApson, KS, Tetrasiklin & Niasinamide, IVIG
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PORPHYRIA CUTANEA TARDA (PCT)
• Disfungsi enzim uroporphyrinogen decarboxylase. • Bula tegang serous/hemorhargis,nyeri +/-• Daerah paparan matahari. • Hipertrikosis, plakat hipopignetasi yang kekuning-
kuningan, alopesia berparut, onikolisis.• Wood lamp urin→merah bata • Porpirin urin ≥ 100μg/24 jam • Bula subepidermal Terapi: -Plebotomy2 kali seminggu→Hb 10μg/dL, kadar besi 50μg/dL - Antimalaria
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SUBCORNEAL PUSTULOSA DERMATOSIS
• Wanita : pria = 4:1
• Extensor, lipatan, inframamae
• Pustul steril tersusun anuler
• Bula subkorneal dengan sebukan neutrofil
• DDS,KS
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HERPES GESTASIONES
• Papuloveskobulosa berkelompok, polimorf
• Kehamilan tm II bulan 5-6 menghilang segera setelah partus
• Bula subepidermal
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PEMFIGOID SIKATRISIAL • Dermatosis bulosa kronik • Bula →sikatrik tu
-Mukosa: mulut (85%), konjunctiva (64%), nasal, nasofaring, faring, esofagus, laring, trachea dan anal. - Kulit: terbatas pada muka, leher, scalp, axila, badan & extremitas.• PA : -Peradang submukosa dengan infiltrat yang padat -Fibrosis submukosa pada upper papillary.• Terapi: -KIL - Bedah - Tracheostomy - IVIG - DDS, MTX, Cyclophosphamide, Chlorambucil, Azathioprine, Mycophenolate mofetil.
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EPIDERMOLISIS BULLOSAKlasifikasi dibagi 2:1. Epidermolisis bulosa tanpa jaringan parut 2. Epidermolisis bulosa dengan jaringan parut
Diduga defisiensi enzim galactosylhydroksylisyl glucosyl tranferase
Predileksi pada daerah yang mudah mengalami trauma Bula epidermis atau dermis
Terapi : - Menghindari trauma - Menusuk bula dengan jarum steril - Makanan yang lembut - Vitamin E 600-1600 iu/hr, difenilhidatoin 2,5-5,0 mg/kg BB/hr.
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TERIMA KASIH
TERIMA KASIH
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Pemfigus vulgaris
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Pemfigus vulgaris
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Pemfigoid bulosa
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Pemfigoid bulosa
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Pemfigoid siktrisial
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D Herpetiformis
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Linear IgA dermatosis