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EMERGENCY CASES IN DERMATOLOGY. Oki Suwarsa Department of Dermato-Venereology Medical School of Padjadjaran University. 1. Stevens Johnson’s Syndrome 2. Toxic Epidermal Necrolysis 3. Pemphigus vulgaris 4. SSSS. STEVENS-JOHNSON’S SYNDROME. Definition : Syndromes Skin

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emergency cases in dermatology

EMERGENCY CASES IN DERMATOLOGY

Oki Suwarsa

Department of Dermato-Venereology Medical School of Padjadjaran University

slide2

1. Stevens Johnson’s Syndrome

  • 2. Toxic Epidermal Necrolysis
  • 3. Pemphigusvulgaris
  • 4. SSSS
stevens johnson s syndrome

STEVENS-JOHNSON’S SYNDROME

Definition :

Syndromes Skin

Orificial and ocular mucosa

Varied general state: mild --- severe

Synonym : erythema multiforme mayor

Etiology :

Systemic drug-induced allergy

Infection

Neoplasm

Pathogenesis :

type I-IV

slide4

DIAGNOSIS :

  • Mild severe decreased consciousness soporous coma
  • TRIAD :
      • Skin disorder
      • Orificial mucous disorder
      • Opthalmic disorder
  • Skin : erythema , papules, vesicles, bullae, erosion, purpura
  • Mucosa :
  • Mouth : 100%
  • Genitalia : 50%
  • Anus/nose : 4 - 8 %
slide5

Eye Disorder :

      • 80% conjuctivitis catarrhalis
      • Corneal ulcer
      • Iritis, Iridocyclitis
  • Complication :
      • Bronchopneumonia
      • Loss of body fluid shock
      • Eye blindness, lacrimation
slide10

THERAPY :

      • Care,General state, Infuse
      • Corticosteroid life saving
      • 4-6 x 5 mg/day critical period (3days)
      • Get decreased to be 5mg/day per day 1x5 mg
      • Antibiotics (carefully)
  • PROGNOSIS :
  • Take action immediately,and appropriately good prognosis
  • Death 5 – 15 %
toxic epidermal necrolysis

Toxic Epidermal Necrolysis

Oki Suwarsa

Bagian Ilmu Kesehatan Kulit dan Kelamin

Fakultas Kedokteran Universitas Padjadjaran

RSUP. Dr. Hasan Sadikin

Bandung

definisi
DEFINISI
  • Toxic Epidermal Necrolysis(TEN)/Lyell’s syndrome
    • Keadaan mengancam jiwa di bidang dermatologi
    • Mengenai kulit, mukosa, &

organ dalam

    • Disertai gejala konstitusi
    • Sering disebabkan alergi obat
    • Kelainan kulit: bula, epidermolisis

& erosi

epidemiologi
EPIDEMIOLOGI
  • Insidensi TEN: 0,4-1,2 kasus per juta populasi pertahun
  • SJS : SJS-TEN : TEN = 3 : 2 : 1
  • TEN tersebar luas di seluruh dunia
  • ♀ : ♂ = 2 : 1
  • Terutama pada dewasa, jarang pada anak
slide15
Bagian Ilmu Kesehatan Kulit dan Kelamin RSUP Dr.Hasan Sadikin Bandung tahun 2008 :

9 penderita SJS-TEN & TEN

    • Semua dewasa, usia 15-72 tahun
    • 6 ♂ & 3 ♀
    • Insidensi pada HIV-AIDS 
etiologi
ETIOLOGI
  • Reaksi terhadap obat (80-95% kasus)
    • Obat tersering penyebab TEN :

Sulfonamid, antikonvulsi, NSAID

    • Kelompok kedua tersering :

Antimalaria, allopurinol dan lain-lain

  • Infeksi
  • Vaksinasi
  • Graft-versus-host disease
  • Idiopatik (<5%)
etiologi1
ETIOLOGI
  • Mencari penyebab pasti TEN sulit:
    • Diagnostik invitro, skin test tidak menolong
    • Tes invivo tidak dilakukan karena etik
  • Etiologi:
    • Anamnesis
    • Bukti-bukti empiris
obat obat penyebab sjs ten
High risk

Allopurinol

Sulfamethoxasole

Sulfadiazine

Sulfapyridine

Sulfadoxine

Sulfasalazine

Carbamazepine

Lamotrigine

Phenobarbital

Phenitoin

Phenylbutazone

Nevirapine

Oxicam NSAID

Thiacetazone

Obat-obat penyebab SJS-TEN
obat obat penyebab sjs ten1
Lower risk

Acetic acid NSAIDs (Diclofenac)

Aminopenicillins

Cephalosporins

Quinolons

Cyclins

Macrolides

Dapat menjadi penyebab

Parasetamol (acetaminophen)

Pyrazolone analgesics

Corticosteroids

Other NSAIDs (except aspirin)

Setraline

Obat-obat penyebab SJS-TEN
obat obat penyebab sjs ten2
Obat-obat penyebab SJS-TEN
  • Belum terbukti sebagai penyebab
    • Aspirin
    • Sulfonylurea
    • Thiazide diuretics
    • Furosemide
    • Aldactone
    • Calcium channel blockers
    • ß Blockers
    • Angiotensin-converting enzyme inhibitors
    • Angiotensin II receptor antagonists
    • Statins
    • Hormones
    • Vitamins
data rsup dr hasan sadikin tahun 2008
Data RSUP. Dr. Hasan Sadikin tahun 2008
  • Obat-obatan yang diduga menyebabkan SJS overlapping TEN dan TEN pada 9 penderita (5 SJS-TEN & 4 TEN):
  • Karbamazepin & parasetamol  terbanyak (5 kasus)
    • Karbamazepin
    • Allopurinol
    • Parasetamol
    • Amoksisilin
  • Ibuprofen
  • Siprofloksasin
  • Fenobarbital
  • Khloramfenikol
slide22
Sulfa & co-trimoxazole  paling banyak

1-10 / 100.000 (rasio relatif 172)

  • Obat-obat antikonvulsan:

karbamazepin, fenitoin dan barbiturat (rasio relatif 11-15)

  • Karbamazepin  sering  14 / 100.000
  • Hidantoin  sebab utama pada anak
  • Negara dunia ketiga antituberkulosa
  • Tahun-tahun terakhir  nevirapin 
patogenesis
PATOGENESIS
  • Reaksi sitotoksik  destruksi keratinosit yang telah ditempel antigen (obat)
  • Onset : 1-45 hari (± 14 hari)
  • Paparan berulang  lebih singkat
  • Obat  mengaktivasi sel T (CD4+ & CD8+)  IL-5
  • Epidermal injury  dasar apoptosis
  • Cairan bula  limfosit T CD8+  & TNF-α
patogenesis1
PATOGENESIS
  • TNF-α  destruksi epidermis
    • Induksi apoptosis secara langsung
    • Menarik sel efektor sitotoksik
  • Makrofag & keratinosit  sumber TNF-α
  • Obat (hapten) + protein  antigen  menempel pada permukaan keratinosit  dimodifikasi menjadi peptida  dipresentasikan oleh MHC-I & MHC-II
  • Fas (Ag) pd keratinosit berikatan dg Fas Ligand  Apoptosis
manifestasi klinik
Gejala prodormal tidak spesifik

(hari ke 1-14) umumnya 3 hr sebelum manifestasi pada kulit

Panas badan

Malaise

Nyeri kepala

Rhinitis

Batuk

Nyeri menelan

Nyeri dada

Mual

Muntah

Diare

Mialgia

Atralgia

MANIFESTASI KLINIK
manifestasi klinik1
MANIFESTASI KLINIK
  • Onset tiba-tiba, (1-45 hari)

umumnya 7-21 hari setelah minum obat

  • Pada pengulangan obat
    • Dapat terjadi dalam 2 hari,
    • Menyebar generalisata,
    • Mengenai mukosa,
    • Disertai gejala konstitusi
    • Keterlibatan organ-organ dalam
manifestasi klinik2
MANIFESTASI KLINIK
  • Kelainan kulit
    • Makula eritem
    • Bula kendur (Nikolsky sign +)
manifestasi klinik3
MANIFESTASI KLINIK
  • TEN
    • menyebar secara cepat dan ekstensif ke seluruh tubuh
    • Berupa eritem difus, bula mudah pecah, epidermolisis, & erosi (epidermolisis 30-90%)
    • TEN berat  kuku jari tangan, kaki, alis, & bulu mata dapat lepas
manifestasi klinik6
MANIFESTASI KLINIK
  • 90 % terdapat Kelainan

pada membran mukosa

(minimal pada 2 tempat)

    • Mukosa mulut: bukal, palatum, vermilion border  hampir selalu terkena
    • Mukosa konjungtiva bulbi & anorektal lebih jarang terkena
  • Pada 40 % mengenai ketiga mukosa
manifestasi klinik8
MANIFESTASI KLINIK
  • Lesi oral 
    • nyeri sekali, sulit makan & bernafas , hipersalivasi
    • Dapat meluas ke ginggiva, lidah, faring, lubang hidung, laring, esofagus & respiratory tree
  • Konjungtiva 
    • vesikulasi, erosi yang nyeri, & lakrimasi bilateral
    • Jarang  konjungtivitis purulen, fotofobia, ulserasi kornea, uveitis anterior, & panoftalmitis
manifestasi klinik9
MANIFESTASI KLINIK
  • Lesi pada genital  lebih sering
    • Gejala bula hemoragik yang nyeri
    • Lesi erosif atau purulen pada fosa navikularis & glans penis atau vulva & vagina
    • Retensi urin & phimosis
manifestasi klinik10
MANIFESTASI KLINIK
  • Organ tubuh lain
    • Paru-paru
    • Saluran pencernaan
    • ginjal
laboratorium
LABORATORIUM
  • Analisa gas darah :
    • Kadar bikarbonat serum < 20 mM  prognosis buruk
  • Kadar elektrolit, albumin, & protein :
    • Ketidakseimbangan elektrolit
    • Hipoalbuminemia
    • Hipoproteinemia
    • Insufisiensi renal ringan
laboratorium1
LABORATORIUM
  • Kadar urea nitrogen darah   penyakit berat
  • Anemia, lekositosis, trombositopenia
  • Neutropenia  prognosis buruk
  • Kadar gula darah, SGOT, SGPT, & amilase dapat 
histopatologi
HISTOPATOLOGI
  • Apoptosis keratinosit pada suprabasal  Epidermolisis
  • Infiltrasi limfosit (limfosit T CD8+) & makrofag yang moderat pada papila dermis
diagnosis banding
DIAGNOSIS BANDING
  • Most likely
    • Epidermolisis terbatas: eritema multiforme mayor, varisela
    • Epidermolisis luas: Acute Generalized Exanthematous Pustulosis (AGEP), Generalized bullous fixed drug eruption
diagnosis banding1
DIAGNOSIS BANDING
  • Consider
    • Paraneoplastic pemphigus
    • Linear IgA bullous disease
    • Pressure blister after coma
    • Phototoxic reaction
    • Graft-versus host disease
diagnosis banding2
DIAGNOSIS BANDING
  • Always rule out
    • Staphylococcal scalded skin syndrome
    • Thermal burns
    • Skin necrosis from disseminated intravascular coagulation or purpura fulminants
    • Chemical toxicity (colchicine intoxication, methotrexate overdose)
komplikasi
KOMPLIKASI
  • Fase akut  sepsis  sebab utama kematian
  • Multy organ failure (MOF)  30%
  • Komplikasi pada paru-paru  15%
  • Komplikasi pada mata  20-75%

(late ophthalmic complication)

    • Fibrosis
    • Entropion
  • Trichiasis
  • Simblefaron
komplikasi1
kuku  50%

Pigmentasi pada nail bed

Dystrophic nails

Anonikia

Komplikasi sering

Dyspareunia

Adhesi genital perlu tindakan bedah

Komplikasi jarang

Striktur esofagus,

Striktur intestinal

Striktur bronkial

Striktur uretral

Striktur anal

KOMPLIKASI
penatalaksanaan
PENATALAKSANAAN
  • Penatalaksanaan tidak hanya dengan obat-obatan, tetapi harus terpadu
  • Stop obat-obat yang dicurigai
    • bila ragu-ragu  semua obat-obatan dalam 8 minggu terakhir yang bukan life saving harus dihentikan
penatalaksanaan1
PENATALAKSANAAN
  • Atasi keadaan umum
    • Bila epidermolisis luas rawat di unit luka bakar atau ICU
    • Atasi keseimbangan hemodinamik & elektrolit
    • Antibiotik profilaksis tidak dianjurkan
    • Mata harus diperiksa setiap hari oleh oftalmologis
    • Mulut dicuci dengan antiseptik kumur
penatalaksanaan2
PENATALAKSANAAN
  • Dapat diberikan kortikosteroid (KS), IVIG, siklofosfamid, siklosporin, plasmaferesis, dan anti TNF  sesuai dengan keadaan.

Pemberian kortikosteroid masih kontroversi

    • Belum ada patokan dosis yang pasti
    • Jerman & Jepang  kortikosteroid first line therapy
slide47
Jepang :
    • Metil prednisolon 80 mg atau 1 gr  diturunkan 70 mg, 60 mg, 40 mg, 30 mg, 20 mg, stop
    • Bila tidak responsif  IVIG 20 gr atau lebih/hari selama 3 hari

atau plasmaferesis

penatalaksanaan3
PENATALAKSANAAN
  • Kepustakan lain 
    • Metil prednisolon 1-2 mg/KgBB/hari

Atau 3-4 mg/kgBB/hari

  • Bagian Ilmu Kesehatan Kulit dan Kelamin FK UNPAD/RSHS 
    • KS pada TEN dimulai dengan deksametason IV 40 mg, yang kemudian diturunkan cepat 30 mg  20 mg  10 mg  5 mg   stop
  • Alternatif terapi :
    • Siklosporin, siklofosfamid, dan anti TNF 
prognosis
PROGNOSIS
  • Mortalitas
    • SJS  5-12%
    • TEN  > 30%
  • SCORTEN  dinilai pada hari ke 3  sebagai acuan
pemphigus vulgaris
Pemphigus vulgaris

Definition :

  • Is a chronic blistering skin disease characterized with epidermal flaccid blisters on the skin and mucous membrane caused by acantholysis process.
  • The blisters occure any where on the skin surface, ussually arises on the normal skin appearance
  • Penyakit kulit dengan tanda adanya bula epidermal yang kronik, dapat terjadi pada kulit dan mukosa. Warna eritem yang terletak pada kulit normal.

31

epidemiology
Epidemiology
  • Male = female, most of the cases present in decade of 5 to 6
  • The children are rare affected

32

internal causal etiology external etiology
Internal (Causal) etiologyExternal etiology

Etiology

- The causal etiology was unknown, and now highly suspicious caused by auto immunity to the inter cellular substance

- External causes noted by some researchers were caused by various drugs:

Penicillamine, Sulfhydril group, Catopril, Penicillins , Rifampin, etc

clinical pictures gambaran klinik
Clinical pictures :(GAMBARAN KLINIK)
  • flacid blisters, eroded and leave hyperpigmented crusts, bad odor smell

(Vesikel atau bula kendur, mudah pecah, erosi, krusta bekas hiperpigmentasi )

  • If the blisters located in the oesophageal mucous membrane, some swallowing disturbance will be present and painful

(Mukosa mulut, oesofagus, disertai gangguan menelan)

33

immunopathogenesis
Immunopathogenesis
  • Caused by autoimmune reaction, the presence of pemphigus antibody in the sera by IF and DIF to keratinocyte surface protein is a hallmark for diagnosis.
    • Antibodi mengikat glikoprotein permukaan sel  Aktivasi plasmin + enzim  merusak segmen interseluler
    • Peranan komplemen dapat dilihat dengan meningkatnya C-3 pada epidermis
predilection of the lesion
Predilection of the lesion:
  • Oral mucous membrane, scalp, trunk, buttocks, head and neck regions, groin and axilla, genital area
  • Predileksi, mukosa mulut, kulit kepala berambut, badan, bokong, muka, leher, ketiak, daerah genitalia
immunopathogenesis1
Immunopathogenesis
  • Caused by auto immune reactions to the inter cellular substances. The auto antibody of IgG or IgM reacts to tho surface glycoprotein of the cells, and activated the plasmin and some proteolitic enzymes, the final results is the destructions of the intercellular attachment of the keratinocytes and microscopic seen as acantholysis of the epidermis.
  • The role of complemen often noted by the increase of C-3 component in epidermal.

34

sign and symptom pemeriksaan penunjang
Sign and symptomPemeriksaan Penunjang
  • Nikolsky Sign (+) or spreading phenomenon
  • Histopathology (Microscopic examination):
    • Acantholytic cells ( +) by Tzanck’s methods)
    • Intraepidermal blisters (bullae)
    • Mostly IgG located at dermo-epidermal junction (imunofluoresence)

35

management penatalaksanaan
ManagementPenatalaksanaan
  • Severe conditions especially suffered with infection, the patients most be admitted in to the hospital and treats as emergency skin disease.
  • Steroid (Prednison as a starting treatment at 400 mg of prednison day).
  • The use of immunosupressant are widely accepted.

39

prognosis1
Prognosis
  • After steroid era the five years survival rate more than 80%
  • Before steroid treatment, fatality rate is very high more than 80 %
  • Quo ad functionam, dubia
  • Quo ad sanationam, dubia
treatment
First choice Treatment:

Steroid treatment and if nescesery combined with immuno supresant drugs to reduce the dosis of systemic steroid.

Second treatment:

Palliative treatment

Supportive treatment

Treatment
bullous pemphigoid
Bullous pemphigoid

Definition :

  • A chronic blistering disease , the blisters laid on the subepidermal area. Usually occur in elderly and characterized by tensed blister and large.
  • The finding of IgD and C-3 at the epidermal basement membrane is a pathognomic sign

42