PENYAKIT KULIT VESIKOBULOSA DAN ERITROSKUAMOSA

PENYAKIT KULIT VESIKOBULOSA DAN ERITROSKUAMOSA

  Nur Hidayat Bagian/SMF Ilmu Kesehatan Kulit dan Kelamin Fakultas Kedokteran dan Ilmu Kesehatan Universitas Tadulako/RSUD Undata

PENYAKIT VESIKOBULOSA DEFINISI PENYAKIT VESIKOBULOSA

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

  

Vesikel : penonjolan kulit dengan batas tegas, berisi cairan serous

dan diameternya < 1 cm, jika diameter > 1 cm disebut bula.

  Keterangan

  

PATOFISIOLOGI TERJADINYA VESIKEL & BULA

Spongiosis:

  Disebabkan karena masuknya cairan dan leukosit dari dermis  edema interselular di antara sel-sel keratinosit  ikatan antar sel melebar  ikatan putus. Contoh:

  dermatitis kontak alergi (DKA)

  

PATOFISIOLOGI TERJADINYA VESIKEL & BULA

Degenerasi balon:

  Disebabkan karena infeksi intraseluler  degenerasi balooning  lisis sel  celah  vesikel/bula.

  Contoh: varicela,

  herpes simpleks

  PATOFISIOLOGI TERJADINYA VESIKEL & BULA 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

  

PATOFISIOLOGI TERJADINYA VESIKEL & BULA

Sitolisis

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

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

  5. Campak

MACAM PENYAKIT VESIKOBULOSA PADA

  Herediter Epidermolisis bulosa Lain-lain

Eritema multiforme

Sindroma Stevens Johnson Nekrolisis Epidermal Toksik

  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 mendahului (minggu – tahun) sebelum pada kulit.

  Pemfigus vulgaris…

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

  Nikolsky sign (+)

  Pemfigus vulgaris… Patogenesis

  Antibodi Ig G (+ komplemen) terhadap desmoglein 3  akantolisis

  Diagnosis banding:

• Pemfigoid bulosa
• Dermatits herpetiformis
• Stomatitis aphtosa
• Eritema multiforme
• Liken planus erosiva
• Pemfigoid membrana mukosa

  Pemfigus vulgaris… Pemeriksaan:

• Pemeriksaan Tzanck : adanya sel akantolitik
• Patologi Anatomi : cleft suprabasal & akantolisis
• Direct immunofluorescence (DIF) : IgG dengan/tanpa komplemen dg pola seperti jala (netlike pattern)
• Indirect immunofluorescence (IIF) : circulating IgG pada 90% pasien

  Terapi:

• Steroid sistemik (Prednison 2-3 mg/Kg BB)
• Imunosupresif (azatioprin, siklosporin, siklofosfamid, mikofenolat mofetil)
• Plasmaferesis
• Imunoglobulin intravena

  PEMFIGOID BULOSA Gambaran klinis

• Usia 60 – 80 tahun
• Bula sub-epidermal, dinding tegang, kadang hemoragis, kadang gatal
• Tanda N ikolsky (-)

  N ikolsky (-)

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

  PEMFIGOID BULOSA Patogenesis

  Antibodi IgG (+ komplemen) terhadap BP antigen (BP 230/BP1 dan BP180/BP2)  bula

  Pemfigoid bulosa… Diagnosis banding

• Dermatitis herpetiformis
• Pemfigus vulgaris

  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.
• Indirect immunofluorescence (IIF) : circulating IgG dengan target komponen membran basal.

  Pemfigoid bulosa… Terapi :

  Steroid sistemik (Prednison1 mg/Kg BB) Azatioprin, mikofenolat mofetil Dapson Tetrasiklin + nikotinamid Steroid topikal pada kasus ringan

  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 enteropathy.

  Dermatitis herpetiformis…

  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

  LINEAR Ig A DERMATOSES

• Papul, vesikel, bula, berkelompok, simetris.
• Bula subepidermal.
• Rasa gatal.
• Predileksi : ekstremitas ekstensor, pantat, mukosa oral & konjungtiva.
• Patogenesis : antibodi

  (IgA) terhadap protein

  97 Kda di membrana basal.

  Linear IgA dermatoses…

• Diferensial diagnosis : Dermatitis herpetiformis
• Terapi :
• Dapson • Sulfapiridin • Steroid dosis rendah

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

  primary herpetic gingivostomatitis infection, primary herpetic gingivostomatitis, herpes labialis, herpetic whitlow, aseptic meningitis, HSV encephalitis Imunokompromais : widespread local infection, chronic ulcers, disseminated cutaneous & visceral infection. 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 (

  HSV-2 : Imunokompeten : primary infection, herpes genitalis, primer & rekuren, herpetic whitlow, aseptic meningitis Imunokompromais : widespread local infection, chronic ulcer, disseminated cutaneous & visceral infection

  Herpes simpleks…

INFEKSI SEKUNDER ).

  Herpes simpleks…

  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)

  VARICELLA

• Usia : kebanyakan anak- anak
• Papul, vesikel, pustul, krusta
• Predileksi : wajah, badan, ekstremitas, mukosa mulut, hidung, konjungtiva, faring, laring, trakea, gastrointestinal, urinaria, vagina.
• Penyebab : virus varicella- zoster

  Varicela…

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

   Obat (sulfonamid, fenitoin, barbiturat, fenilbutazon, penisilin, alopurinol)  Infeksi (herpes simpleks,

  ERITEMA MULTIFORME • Usia : 50% di bawah 20 th.

• Papul eritem iris-shape & lesi vesikobula.
• Penyebab :

  Mycoplasma)  Idiopatik • Predileksi : ekstremitas

  (telapak tangan & kaki) & membrana mukosa (mulut,

  Eritema multiforme… Diagnosis banding:

• Fixed Drug Eruption •Primary herpetic ginggivosto- matitis

  Terapi :

  Tergantung penyebab :

• HSV  antiviral
• Mycoplasma  antibiotik macrolid
• Topikal steroid

SINDROMA STEVENS JOHNSON DAN

• 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).
• TEN : ditambah bahan kimia,

  Mycoplasma pneumonia, viral,

  SJS & TEN…

• 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.

  Selesai Selamat belajar

  

Kelompok penyakit kulit dengan lesi utama

patch, plak atau papul eritematosa dengan skuama

PSORIASIS PITYRIASIS ROSEA PITYRIASIS RUBRA PILARIS LICHENOID ERUPTION

  

DERMATITIS EKSFOLIATIVA/ERYTHRODERMIA

etc

  PAPULA:

PENONJOLAN KULIT PADAT UKURAN<0,5 CM

PENGELUPASAN KULIT BERLEBIHAN. SKUAMA: TAMPAK./ PATOLOGIS

  

UNKNOWN CAUSE

  _ (MULTIFACTORIAL) _ CHRONIC AND RECIDIVE COURSE ERYTHROSQUAMOUS PLAQUE

WITH MICACEOUS MULTILAYERD SCALE

• CONTINOUS WOUND HEALING TYPE OF

KERATINOCYTE HYPER PROLIFERATION

• CONTINOUS INDUCTION BY INFLAMATORY CELL

• • SHORTHERNING OF KERATINOCYTE TURN OVER

  PATHOGENESIS OF PSORIASIS Triggering factors



emotional stress ENVIRONMENTAL FACTORS

   season PHYSICS,BIOLOGIC AND

   trauma EMOTIONAL

   drugs

   infection PSORIATIC SKIN PREPSORIATIC SKIN genetic :

  IMUNOLOGIC AND NON IMUNOLOGIK HLA DR. BW PROCESSES diet

  

Schematic representation of generating psoriasis inflammatory

lesion from normal psoriatic skin. Starting from activation of native

APC’s to the maintenance of psoriatic lesion

  Adapted from Prinz, 2003

HISTOPATOLOGICAL FINDING

  

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

PSORIASIS VULGARIS

  COMMONEST FORM , SPECIFIC ERYTHROSQUAMOUS PLAQUE OVER PROMINENCE AREA OF THE SKIN

  Koebner phenomen ( isomorphic ) isomorphic eruption following repetitive trauma ( LESIONS ARRANGED IN A LINE) Wax drop phenomen The micaceous scale be- coming dull if scratched

  Auspitz sign pin point bleeding when pull off the scale

HISTOPATOLOGICAL FINDING

  

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

  FLEXURAL / INVERSE PSORIASIS SPECIFIC PLAQUE AT FLEXURAL AREA SEBORIASIS - PSORIASIS SEBORHEIC

  GUTTATE PSORIASIS ERUPTIVE MULTIPLE, SMALL SIZE DROP LIKE LESIONS >> IN CHILDREN AS NEW LESIONS

  

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 some time happen together with the plaque type

  

PSORIASIS ARTHROPATHY

arthritis seronegative
• predilection: small joint
• persisten

• •specific Radiologic pattern

  Psoriasis of the nail >>hand nails  pitting nail  yellowish discoloration  subungual hyperkeratosis  lateral onycholysis

  _ 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 Sosialization of the illness to the patient and family

  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 Lymphadenopathy, ectropion of the eyelid, edem at lower extremities, some times fever, hypoproteiaemia,

• ⁺⁺ leucocytosis, depletion [Ca] serum.

  Psoriasis erythrodermia

• reactive psoriasis
• exfoliation > 75% body surface

BEFORE TREATMENT

  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 (corymbi form pattern)

  clinical course start as single lesian accompanied with other sall lesions Herald patch generalized --- > faded Herald patch, close up

  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.

  

Therapy: symptomatic, anti inflammatory drugs ( steroid)

erythomycin systemic, UV-B photo therapy

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)
• VIT.A DEFFICIENCY ?

  LESION ON THE PALM AND SOLE Keratotic sandal Yellowish colour Diffuse hyper keratosis on palms The nail changes not specific

  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

  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

  INFECTION -PSYCHOGEN-GENETIC ENZYMATIC IMMUNOLOGIC

CLINICAL MANIFESTATION

  PAPULE, SINGLE LESION : FLAT TOPPED POLYGONAL WITH WICKHAM STRIAE, DEEP PURPLE HUE DISCRETE OLD LESION VARIED MUCOSAL LESION : WHITISH PLAQUE (LACY WHITE APPEARANCE)

  Koebner sign Flat topped polygonal lesion with striae of Wickham