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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