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GANGGUAN PIGMENTASI
Rosmelia
Departemen Ilmu Kesehatan Kulit danKelamin FK UII
Yogyakarta
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WARNA KULIT
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Carotenoid
DeoxyHBOxyHB
SKIN
COLOR
Melanosome degradation
Melanosome stages & sizes
Tyrosinase synthesis
Epidermal melanin unit
Melanosome transfer & distr
kolagen
Melanin
Warna kulit
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Epidermal Melanin Unit
Kepadatan : 550 - >1200/mm2Terpadat di : wajah dan genitalia
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TYROSINE
Tyrosinase
L-DOPA
DOPAquinone
DHI
DOPAchrome
CysteinylDOPA
Tyrosinase
Indole 5,6-quinone
DHI melanin
Black, insoluble
High MW
TRP-2DHICA
Indole 5,6-quinonecarboxylic acidTyrosinase or
TRP-2
DHICA melaninBrown, poorly soluble
Intermediate MW
Glutathione orcysteine
Alanyl-hydroxy-benzothiazine
Pheomelanin
Red/yellow
Soluble, low MW
Tyrosinase
UV
POMC (MSH&ACTH),IL-1, PGE2, GM-CSF,TNF , NO, bFGF
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Regulasi biosintesis melanin
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Endothelin (ET-1) Produced by UV irr-keratinocytes Up-regulated MC1R Melanocytes proliferations
Prostaglandin and leucotriens Melanocytes proliferations Melanocytes migrations
Histamine Tyrosinase levels Melanocytes dendricity
Neurothophins Produced by UV irr-keratinocytes Inhibit melanocytes death
- FGF Produced by UV irr-keratinocytes
and fibroblasts Mitogen for keratinocytes
KGF Produced by UV irr-keratinocytes Transfer melanosomes
Melanin Biosynthesis
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MACAM-MACAMGANGGUANPIGMENTASI
HIPOPIGMENTASI
Melanositopenik Melanopenik
Kongenital Piebaldisme Albinisme
Didapat Vitiligo,leukodema
kimia/obat
Hipopigmentasi
pascainflamasi,
Pitiriasis alba,PVC
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VITILIGO
Ditandai dengan makula/patch berbatas tegas berwarnaputih susu yang tidak memiliki melanosit
Insidensi : antara 1-2%, kadang familial, kadang terkaitpeny. Sistemik (Tiroiditis Hashimoto, peny Graeves)
Tipe klinik:- Fokal: terbatas jumlah dan ukuran- Segmental: unilateral, dermatomal/quasi dermatomal- Generalisata (simetris bilateral): tersebar pada keduasisi tubuh, biasanya di permukaan ekstensor,
-Akrofasial: distal jari2 dan wajah- Universal
Predileksi: wajah (terutama peri-orifisium), dada atas,aksila, selangkangan
Dapat mengalami fenomena Koebner
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PATOGENESIS
Teori:
1. Mekanisme autoimun: destruksi melanosit dimediasioleh sel T autoreaktif
2. Mekanisme neural: akhiran saraf melepaskan mediatorkimiawi yang bersifat toksik terhadap melanosit
3. Mekanisme autositotoksik/metabolik: radikal bebas yangdihasilkan oleh sel menyebabkan kerusakan melanosit
DD: PVC, Pitiriasis alba, hipopigmentasi pascainflamasi,leukoderma kimiawi
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TERAPI
Tabir Surya
Topikal: kortikosteroid, calcineurin inhibitor(tacrolimus,pimecrolimus), 8-metoksipsoralen oral/topikal + UVA(PUVA), NB-UVB
Bedah: minigrafting, suction-blister grafting, transplantasi
suspensi sel epidermal autolog
Depigmentasi: monobenzyl ether of hydroquinone
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ALBINISME(Oculocutaneus albinism)
Merupakan penyakit herediter ditandaidengan defisiensi pigmen melanin padamata, kulit dan rambut o.k.
Imaturitas melanosom. Tipe: OCA1, OCA2, Prader-Willi and
Angelman Syndromes, OCA3, OCA4,Hermansky-Pudlak syndromes, Griscelli
syndromes, Chediak-Higashi syndrome Mutasi pada gen: TYR, P, TYRP1,
MATP, HPS, LYST diwariskanautosomal resesif
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HIPOPIGMENTASI PASCAINFLAMASI
Mekanisme tidak diketahui
Bentuk makula/patchhipopigmentasi / depigmentasi
Hipopigmentasi: pasca dermatitisatopik, PVC
Depigmentasi: pasca peradanganberat (LE), trauma (mis. Lukabakar), bahan kimia toksik (mismonobenzylether of hydroquinone,p-phenylendiamine,p-tertiarybutylphenol)
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HIPERPIGMENTASI
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EFELID/FRECKLES
Makula coklat tua/coklatmuda/merah, multipel, ukuran 1-3 mm, daerah sun-exposed
Muncul pada masa anak dewasa.
Faktor genetik: melanosit normal,melanosom dan aktivitasmeningkat autosomal
dominan
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MELASMA
Kloasma, mask of pregnancy
Hiperpigmentasi warna coklatyang muncul pada daerahterpapar matahari
Berkaitan dengan kehamilan,hormon, obat difenilhidantoin,genetik
Melanin epidermal meningkat,jumlah melanosit meningkat
Tipe: sentrofasial, malar,mandibular
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HIPERPIGMENTASIPASCAINFLAMASI
Hiperpigmentasi pada lokasiperadangan sebelumnya
Epidermal atau dermal
2 mekanisme:- Meningkatnya aktivitas
melanosit (karena sitokinproinflamasi)
- Keluarnya melanin karenarusaknya melanosit
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Process Agents
Before melanin s ynthesis
Tyrosinase transcription inhibition Tretinoin
Dur ing melanin synthesisTyrosinase inhibition Hydroquinone, arbutin, aloesin,
azelaic acid, kojic acid, licorice extract-glabridin
Peroxidase inhibition Phenols
Product reduction and ROSscavengers Ascorbic acid
Terapi pada kelainan hiperpigmentasi
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Process AgentsAfter melanin sy nthesis
Tyrosinase degradation Linoleic acid, -linolenic acid
Inhibition of melanosome transfer Soybean/milk extracts, lecithins ,niacinamide
Skin turnover acceleration Glycolic acid, lactic acid, linoleic acid,tretinoin,
Others Sunprotection, Corticosteroids
Terapi pada kelainan hiperpigmentasi
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Terapi pada kelainan hiperpigmentasi
Terapi lain: Chemical peeling
Laser
Mikrodermabrasi