REFERAT

BULLOUS DISEASE
{ DISUSUN OLEH : Rizka Aulia Hermawati
PEMBIMBING : dr. Bowo Wahyudi,Sp.KK

KEPANITERAAN KLINIK ILMU KESEHATAN KULIT & KELAMIN

RUMAH SAKIT UMUM DAERAH BANJAR
PERIODE 23 OKTOBER 26 NOVEMBER 2017
FAKULTAS KEDOKTERAN UMJ
 Genetik Autoimun

Epidermolysis
Pemfigus
bullosa

Skin fragility
Pemfigoid
syndrome

Hailey-hailey
syndrome

BULLOUS DISEASE
Genetik
Epidermolysis Bullosa
• Dowling Meara (severe)

EBS • Kobner (moderate)

• Weber-cockayne (mild)

JEB • Herlitz
• Non Herlitz

• Hallopeau-siemens recessive

DEB • Non Hallopeau-siemens recessive

• Autosomal dominant
 EBS

DEB

JEB Herlitz
Skin Fragility •Resesif autosomal
Syndrome •Kelainan gen plakophilin

Hailey-hailey •Dominan autosomal

Disease •Mutasi gen ATP2C1
Autoimun
 BULA INTRAEPIDERMAL
Pemfigus • KU(buruk), Bula kendur, Nyeri (tdk gatal)
• Kortikosteroid, kurangi aktivitas dg risiko cedera
Vulgaris kulit

Pemfigus • Eritema menyeluruh, byk skuama kasar, krusta

• Mutasi gen ATP2C1
foliaceus
P. Vegetans • Px: muka, aksila, genetalia eksternal, lesi oral
• Bula kendur erosi
Neumann

P. Vegetans • Kronik
• Pustul yg bersatu meluas ke perifer
Hallopeau
 • KU(baik), eritema berbatas tegas, skuama, krusta
Pemfigus (menyerupai kupu2)
eritematosus • Kortikosteroid

• Erosi, ulserasi dan krusta hemoragik di

Pemfigus membrane mukosa
paraneoplastic • Kortikosteroid
Pemfigus drug • Thiol dan non thiol
induced • Hentikan obat2an

Pemfigus
• Bayi baru lahir dg ibu pemfigus
neonatal

• e/ endapan IgA di epidermis

Intercelullar
• Vesikel dan pustula (badan dan
IgA dermatosis ekstermitas
Autoimun
 BULA SUBEPIDERMAL
Pemfigoid • Seluruh tubuh
• Bula tegang, gatal,
bullosa

Pemfigoid • Kehamilan 4 bulan – postpartum

• Badan, bokong, ekstremitas
gestationis • Papulo-vesikel, gatal
 Pemfigoid •Rongga mulut, ocular mucosa
sikatrisial •Bula, erosi

Epidermolysis •Siku, lutut

bullosa acquisata •Bula, erosi
 Dermatitis • Seluruh tubuh
herpetiformis • Gatal, vesikel tersusun berkelompok

Linear IgA bullous • Seluruh tubuh

dermatosis • Eritema, bula tegang, gatal
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