PENYAKIT KULIT VESIKOBULOSA DAN ERITROSKUAMOSA

(1)

PENYAKIT KULIT VESIKOBULOSA

DAN

ERITROSKUAMOSA

Nur Hidayat

Bagian/SMF Ilmu Kesehatan Kulit dan Kelamin Fakultas Kedokteran dan Ilmu Kesehatan

(2)

Kelompok penyakit yang ditandai dengan gejala utama erupsi vesikel dan bula.

PENYAKIT VESIKOBULOSA

DEFINISI PENYAKIT VESIKOBULOSA

Vesikel : penonjolan kulit dengan batas tegas, berisi cairan serous dan diameternya < 1 cm, jika diameter > 1 cm disebut bula.

Vesikel Bula

(3)

PATOFISIOLOGI TERJADINYA VESIKEL & BULA

Spongiosis:

Disebabkan karena masuknya cairan dan leukosit dari dermis  edema interselular di antara sel-sel keratinosit

(4)

PATOFISIOLOGI TERJADINYA VESIKEL & BULA

Degenerasi balon: Disebabkan karena infeksi intraseluler 

degenerasi balooning

 lisis sel  celah 

vesikel/bula.

(5)

Akantolisis:

Disebkan karena proses akantolisis, yakni

hilangnya spina atau akanta atau jembatan antar sel, sehingga terbentuk celah atau rongga yang berisi cairan.Biasanya

disebabkan autoimun. Contoh : pemfigus

(6)

Sitolisis

Disebabkan kerusakan/ketidaksempurnaan komponen sel/ struktur lapisan kulit  sitolisis  sehingga terbentuk celah. Contoh : epidermolisis bulosa

(7)

MACAM PENYAKIT VESIKOBULOSA PADA

Diperantarai imunologi _{1. Pemfigus vulgaris}

2. Pemfigoid bulosa

3. Dermatitis herpetiformis 4. Linear IgA dermatoses 5. Herpes gestasiones 6. Cicatricial pemphigoid 7. Pemfigus paraneoplastik

Virus 1. Herpes simpleks 2. Varicella

3. Hand, foot & mouth disease 4. Herpangina

(8)

Herediter Epidermolisis bulosa

Lain-lain _{Eritema multiforme}

Sindroma Stevens Johnson Nekrolisis Epidermal Toksik

(9)

PEMFIGUS VULGARIS

Gambaran klinis

• usia 50 - 60 th

• bula superfisial (supra basal), dinding kendor (flacid), hipopion,

nikolsky sign + (bula cenderung meluas bila kena tekanan), mudah pecah  kulit terkelupas  krusta

• bau khas ~ tikus (mousy odor) • Bula pada rongga mulut

(10)

• predileksi: kulit kepala, wajah, leher, lipatan aksila, inguinal, periumbilikal, genital, mukosa : bibir, buccal, palatum durum et mole, lidah, mukosa alveolar & ginggiva.

Pemfigus vulgaris…

(11)

Diagnosis banding:

•Pemfigoid bulosa

•Dermatits herpetiformis •Stomatitis aphtosa

•Eritema multiforme •Liken planus erosiva

•Pemfigoid membrana mukosa

Patogenesis

Antibodi Ig G (+ komplemen) terhadap desmoglein 3 

akantolisis

(12)

Terapi:

•Steroid sistemik (Prednison 2-3 mg/Kg BB)

•Imunosupresif (azatioprin, siklosporin, siklofosfamid, mikofenolat mofetil)

•Pemeriksaan Tzanck : adanya sel akantolitik

•Patologi Anatomi : cleft suprabasal & akantolisis

•Direct immunofluorescence (DIF) : IgG dengan/tanpa komplemen dg pola seperti jala (netlike pattern)

(13)

• Usia 60 – 80 tahun

• Bula sub-epidermal, dinding tegang, kadang hemoragis, kadang gatal

• Tanda NNikolsky (-)ikolsky (-)

• Predileksi : paha, aksila, lengan, tungkai, perut, mukosa buccal

PEMFIGOID BULOSA

(14)

Patogenesis

Antibodi IgG (+

komplemen) terhadap BP antigen (BP

230/BP1 dan

BP180/BP2)  bula

(15)

Diagnosis banding

• Dermatitis herpetiformis • Pemfigus vulgaris

Pemfigoid bulosa…

Pemeriksaan:

•Patologi Anatomi : cleft subepidermal dengan inflamasi dermis atas, t.d limfosit, histiosit & eosinofil

•Direct immunofluorescence (DIF) : deposisi linear IgG dan komplemen di dermo-epidermal juntion.

(16)

Terapi :

_{Steroid sistemik (Prednison1 mg/Kg BB)} _{Azatioprin, mikofenolat mofetil}

_Dapson

_{Tetrasiklin + nikotinamid}

_{Steroid topikal pada kasus ringan}

(17)

DERMATITIS HERPETIFORMIS

• Usia : 20 – 60 th. • Papul eritem, plak

urtikaria, vesikel, bula, berkelompok, simetris. • Rasa gatal

• Predileksi :

ekstremitas ekstensor, pantat, sakral,

skapula, wajah, kepala.

• Berhubungan dengan gluten sensitive

(18)

Patogenesis : antibodi (Ig A) thdp antigen di usus 

kompleks Ag-Ab kmd

terdapat di kulit, ATAU Ig A berikatan pd antigen di

kulit.Terjadi aktivasi

komplemen & penarikan netrofil rusak jaringan.

Diferensial diagnosis : • Dermatitis kontak alergika • Papular urtikaria

• Pemfigoid bulosa

Terapi :

Dapson 100-150 mg

Sulfapiridin

Gluten-free diet

(19)

LINEAR Ig A DERMATOSES

• Papul, vesikel, bula, berkelompok, simetris. • Bula subepidermal.

• Rasa gatal.

• Predileksi : ekstremitas ekstensor, pantat,

mukosa oral & konjungtiva.

(20)

• Diferensial diagnosis : Dermatitis herpetiformis • Terapi :

• Dapson

• Sulfapiridin

• Steroid dosis rendah

(21)

HERPES SIMPLEKS

• Vesikel berkelompok dg dasar eritem pada kulit/mukosa.

• Usia : kebanyakan dewasa, tp anak s/d tua.

• Penyebab dan manifestasi : HSV-1

Imunokompeten : primary infection, primary herpetic gingivostomatitis, herpes labialis, herpetic whitlow, aseptic meningitis, HSV encephalitis

Imunokompromais : widespread local infection, chronic ulcers, disseminated cutaneous & visceral infection.

(22)

Patogenesis : virus  infeksi

kulit/mukosa tdk intak  replikasi virus di parabasal sel epitelial  lisis sel  vesikel (INFEKSI PRIMER) 

nervus sensori  ganglion

sensori/autonom (laten)  reaktivasi virus  nervus sensori  vesikel

(INFEKSI SEKUNDER).

HSV-2 :

Imunokompeten : primary infection, herpes genitalis, primer & rekuren, herpetic whitlow, aseptic meningitis Imunokompromais : widespread local infection, chronic ulcer, disseminated cutaneous & visceral infection

(23)

Terapi :

Antiviral topikal (asiklovir, pensiklovir)

Antiviral oral (Infeksi primer : asiklovir 5 x 200 mg 7 hr, valasiklovir 2 x 1 gr 7 hr. Infeksi rekuren : asiklovir 3 x 400 mg 5 hr, valasiklovir 2 x 500 mg 5 hr)

(24)

VARICELLA

• Usia : kebanyakan anak-anak

• Papul, vesikel, pustul, krusta • Predileksi : wajah, badan,

ekstremitas, mukosa mulut, hidung, konjungtiva, faring, laring, trakea,

gastrointestinal, urinaria, vagina.

(25)

Terapi :

Antiviral (asiklovir 5 x 800 mg 5 hr, foscarnet 40 mg/KgBB iv.

(26)

ERITEMA MULTIFORME

• Usia : 50% di bawah 20 th. • Papul eritem iris-shape &

lesi vesikobula. • Penyebab :

 Obat (sulfonamid, fenitoin, barbiturat, fenilbutazon, penisilin, alopurinol)

 Infeksi (herpes simpleks, Mycoplasma)

 Idiopatik

(27)

Diagnosis banding: •Fixed Drug Eruption

•Primary herpetic ginggivosto-matitis

Terapi :

Tergantung penyebab : •HSV  antiviral

•Mycoplasma  antibiotik macrolid •Topikal steroid

(28)

SINDROMA STEVENS JOHNSON DAN NEKROLISIS EPIDERMAL TOKSIK

• Usia : dewasa

• Epidermis nekrotik &

mengelupas, Nikolsky +. • Predileksi :

• Wajah, badan, membrana mukosa (bibir, buccal,

konjungtiva, genital, anal. • Penyebab : obat (sulfa,

alopurinol, hidantoin,

karbamazepin, fenilbutazon, piroksikam, penisilin,

sefalosporin, dll).

(29)

• Patogenesis : mekanisme

imunologis  reaksi sitotoksik cell-mediated.

• Diagnosis banding: • Eritema multiforme • Sindroma syok toksik

• Graft-versus-host disease • Terapi :

• Stop obat dicurigai

• Keseimbangan air & elektrolit. • Kortikosteroid sistemik

• Terapi topikal spt luka bakar • Antibiotika.

SJS & TEN…

Selesai

(30)

PSORIASIS

PITYRIASIS ROSEA

PITYRIASIS RUBRA PILARIS

LICHENOID ERUPTION

DERMATITIS EKSFOLIATIVA/ERYTHRODERMIA

etc

(31)

(32)

(33)

 UNKNOWN CAUSE

(MULTIFACTORIAL)

 CHRONIC AND RECIDIVE COURSE  ERYTHROSQUAMOUS PLAQUE

WITH MICACEOUS MULTILAYERD SCALE

BASIC PATHOLOGY OF LESION

• CONTINOUS WOUND HEALING TYPE OF KERATINOCYTE HYPER PROLIFERATION

•CONTINOUS INDUCTION BY INFLAMATORY CELL

(34)

Triggering factors

_{emotional stress}  season

_trauma _drugs _infection

PATHOGENESIS OF PSORIASIS

PREPSORIATIC SKIN

ENVIRONMENTAL FACTORS PHYSICS,BIOLOGIC AND EMOTIONAL

PSORIATIC SKIN

(35)

Schematic representation of generating psoriasis inflammatory lesion from normal psoriatic skin. Starting from activation of native

APC’s to the maintenance of psoriatic lesion

(36)

 DIFFUSE PARAKERATOSIS,

THINING OF SUPRA PAPILARY EPIDERMIS,

 MICRO ABSES MUNRO,

 REGULER ACANTHOSIS,

 FINGER LIKE DERMAL

PAPILLAE

 EDEM OF DERMAL

PAPILLAE,

 SPARSE PERIVASCULAR

LYMPHO HISTIOCYTIC INFILTRATRATION

(37)

COMMONEST FORM ,

SPECIFIC

ERYTHROSQUAMOUS

PLAQUE OVER

PROMINENCE AREA OF

THE SKIN

(38)

Auspitz sign

pin point bleeding when pull off

the scale

Koebner phenomen ( isomorphic

)

isomorphic eruption following

repetitive trauma ( LESIONS ARRANGED IN A LINE)

Wax drop phenomen

(39)

 DIFFUSE PARAKERATOSIS,

THINING OF SUPRA PAPILARY EPIDERMIS,

 MICRO ABSES MUNRO,

 REGULER ACANTHOSIS,

 FINGER LIKE DERMAL

PAPILLAE

 EDEM OF DERMAL

PAPILLAE,

 SPARSE PERIVASCULAR

LYMPHO HISTIOCYTIC INFILTRATRATION

(40)

SPECIFIC PLAQUE

AT FLEXURAL

AREA

SEBORIASIS -

PSORIASIS

SEBORHEIC

(41)

ERUPTIVE

MULTIPLE, SMALL SIZE DROP LIKE LESIONS

>> IN CHILDREN AS NEW LESIONS

(42)

GENERALIZED PUSTULAR PSORIASIS (von zumbusch)

generalized steril pustules, which are eruptive accompanied high fever, WBC > 20.000/ML DECREASE [Ca++] serum

PSORIASIS PUSTULAR

PALMOPLANTAR Pustular Eruption limitted at palmoplantar , recurrence

(43)

PSORIASIS ARTHROPATHY

arthritis seronegative •predilection: small joint •persisten

(44)

>>hand nails



pitting nail



yellowish

discoloration



subungual

hyperkeratosis



lateral onycholysis

(45)

BASIC MEDICAMENTATION _{TOPICAL : TAR, SALICYLIC ACID, STEROID,TACROLYMUS}

_{SYSTEMIC : CYTOSTATIC AGENT (Cy-A, Mtx) , ISOTRETINOIN, “steroid”} _{COMBINATION : Irrad UV-B + tar ( Reg.Gaukerman)}

irrad UV-A + psoralen (PUVA)

Drug considerance

_{diseases severity ( PASI score )}

 treatment history including medication side effects

_{patient situational status}

Social/holistic approach

(46)

DERMATITIS EXFOLIATIVA (DE)

An eruptive skin disease as whole body erythema and scaling.

Cause : several kinds of dermatosis (skin disease) which undergone an eruptive state,ie:

1. Psoriasis/ other erythrosquamous eruption

2. Dermatitis ( Atopik, Seborrheic, Contact Allergic) 3. Drug eruption

4. Malignancy (cutaneous lymphoma) 5. Ichtyosis

8. Bullous disease (Pemphigus foliaceous)

Clinical sign and course

Generalized erythema and scaling

(47)

Psoriasis erythrodermia

• reactive psoriasis

•exfoliation > 75% body surface

BEFORE TREATMENT

(48)

PITYRIASIS

ROSEA

E S E specific oval

erythematous patch, multiple, fine scale , long axis in line with Langer’s line,

spontaneous regression

Etiology : unknown (viral ?)

Herald patch, bigger size surrounded by the new lesions

(49)

clinical course

start as single lesian accompanied with

other sall lesions

generalized --- > faded Herald patch

(50)

Epidemiology: world wide, male/ female equal child/ adult equal

Clinical manifestation:

Herald patch ----> smallest lesion surrounding spread all-over body but face and palm/sole

Full blown on the back ---> tree form appearance Lesions tend to distribute on clothed area.

Atypical forms : vesicular lesions Self limitted in 6 - 12 weeks

cleared lesions left hypo - pigmented patch.

(51)

PITYRIASIS RUBRA PILARIS

• RARE

• SPECIFIC :

ERYTHROSQUAMOUS

PAPULES, ACCUMINATE, FOLLICULAR, TEND TO COALESCE/

CONFLUENCY

• OLD LESION LEAVE

SMALL ISLANDS OF HEALTHY SKIN

ETIOLOGY

• FAMMILIAL (Aut.Domin)

(52)

LESION ON THE PALM AND SOLE

Keratotic sandal Yellowish colour

Diffuse hyper keratosis on palms

(53)

PATHOGENESIS

INCREASE OF EPIDERMO-POIESIS UP TO 2-3 x NORMAL

CLINICAL MANIFESTATION

New Lesion : erythro squamous patches on the head (seborrhoiformis) start with follicular erythematous papules.

Coalescence of lesions

In the palm and sole thick, luminence wax yellow colored (keratodermic -sandal)

Old lesions : islands of normal skin between the plaque

Eruptive form: similar with dermatitis exfoliative of any cause

HISTOPATHOLOGY

• NOT DIAGNOSTIC BUT SPESIFIC

• HYPERKERATOSIS, PARAKERTOSIS ALTERNATING/FOLIKULER

• EUGRANULOSIS

TREATMENT

TOPICAL : STEROID

TAR + UV-B

(54)

LICHEN PLANUS

A COMMON CHRONIC INFLAMATORY SKIN DISEASE WITH SPECIFIC CLINICAL AND HISTOPATHOLOGICAL MANIFESTATION, UN KNOWN CAUSE

CLINICAL VARIATION

ERYTHRO SQUAMOUS, VESICO BULLOUS, HYPER-TROPHIC,

ATROPHIK, ANULAR, FOLLICULAR, ACTINIC OR ERYTHEMATOUS

ETIOPATHOGENESIS

(55)

CLINICAL MANIFESTATION

SINGLE LESION : FLAT TOPPED PAPULE, POLYGONAL WITH WICKHAM STRIAE, DEEP PURPLE HUE

DISCRETE

OLD LESION VARIED MUCOSAL LESION :

WHITISH PLAQUE

(56)

Koebner sign

(57)