iktiosis sle
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slide kuliah ichtyosis sleTRANSCRIPT
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IKTIOSIS VULGARIS
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IKTIOSIS
• Kelompok heterogen penyakit kulit skuama generalisata dan, sering, penebalan kulit
• Skuama prubahan diferensiasi epidermis • Dpt diikuti o/ eritema, abnormalitas bagian
kulit lain dan struktur adneksa• Iktiosis ichthys (Yunani) – fish kesamaan
penampilan kulit dengan sisik ikan• Warisan atau didapat
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GAMBARAN KLINIS
• SKUAMA, dpt diikuti eritema, abnormalitas bagian kulit lain & struktur adneksa
• Hiperkeratosis (penebalan kulit) dengan atau tanpa skuama penebalan stratum korneum
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IKTIOSIS VULGARIS
• Paling sering dan relatif ringan• Autosom semidominan
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• Siemens mutasi gen terkait patofisoligi
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• Usia 1 tahun• Predileksi: >>> permukaan ekstensor
ekstremitas (terutama ekstremitas bawah) skuama putih halus generalisata -- skuama dengan cracking (fisura superfisial) pd tepinya
• Hiperliniar plam, penebalan plantar/palmar (keratoderma), keratosis pilaris, atopi
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• Hipohidrosis dengan intoleransi thdp panas jarang
• Faktor perberat: iklim yg kering dan dingin• Faktor peringan: lingkungan lembab, hangat
bersih
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Mutasi gen pengkode profilaggrin derajat keparahan penyakit --- xerosis – iktiosis vulgaris yg berat
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Pemeriksaaan Histopatologi
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• Filagrin protein epidermal filamen keratin & kelembapan stratum korneum
• Studi biokimia iktiosis vulgaris tidak ada/ ↓ filagrin & prekusornya (profilagrin)
• Kultur keratinosit defek profilagrin
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Tatalaksana• Inherited simtpomatis, hidrasi, lubrikasi,
keratolisis• Hidrasi melunakan permukaan kulit long
bath mempermudah penipisan area hierkeratosis dengan abrasif ringan (sponges, buff puffs, pumice stone, dll) lubrikan (losio, krim, salap, minyak atau petrolatum)
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• Keadaaan lembab/ basah membaik bahkan hilang pd iktiosis vulgaris ringan
• Iklim kering atau dingin pelembab• Keratolitik deskuamasi kulit krim atau losio
-- urea, asam salisilat, asam hidroksin α (asam laktat, asam glikolat) perhatikan penggunaan agen topikal pd area luas krn defek sawar kulit
• Retinoid topikal atau vitamin D
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• Anak-anak perhatikan nutrisi risiko gagal tumbuh
• Intolerasi panas jarang waspada• Retinoid sistemik (isotretionin atau asitretin)• Infeksi jamur kulit dan kuku tidak
terdiagnosis krn skuama generalisata gatal atau alopesia terlokalisasi atau perbedaan karakter skuama
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Lupus Eritematosus
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Lupus Eritematosus
• Penyakit yang menyerang sistem konektif dan vaskular
• 2 varian– Lupus eritematosus diskoid (LED)– Lupus eritematosus sistemik (LES)
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Lupus Eritematosus
• Etiologi & Patogenesis– Interaksi dari host, lingkungan, agen• Host = predisposisi genetik• Lingkungan = UV, obat-obatan, rokok• Agen = EBV?
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Terapi
• Local Therapy– Sun protection ( SPF min. 30)– Local glucocorticoid • Class 1 (clobetasol propionate 0.05%, Betamethasone
dipropionate 0.05%)• Ointment / Gel• Pemakaian 2 minggu rest period 2 minggu• 2 kali sehari.
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Terapi
– Topical calcineurin inhibitor• Pimetrolimus 1%• Tacrolimus 0.05%
– Intralesional glucocorticoid• Triamcinolone acetonide 2.5 – 5.0 mg/ml
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Terapi
• Systemic Therapy– Antimalarial• Hydroxychloroquine (6.5mg/kg/day) 2-3 month delayed
onset• Chloroquine 3 - 3.5 mg/kg/day• Quinacrine 100mg dailly
– Systemic glucocorticoid• Prednisone (single morning dose 20-40mg/day)• Reduce 5-10mg until 20mg/day then 2.5mg/day