TOXIC EPIDERMAL

NECROSIS
TEACHING BANGSAL
GROUP MEMBERS
KHAIRIYAH SURIATMAJAYA
NADHIRAH BINTI MOHD. NOH
WANDRYATMO SANTOSA TONAPA
JIMMY PATABANG
SUDARMAN ARUNG T.
ANDI DWI RAHMAT ARMYN
ANDI ALFISYA BAYU
NURIA IFTITAH DEDIKASIH
ANDI TENRI ISMI SHANDRA
RACHMAT HIDAYAT
PATIENTS IDENTITY
Name: Ms.Sarmina
Gender: Female
Age: 40 y.o
Marital Status: Married
Religion: Islam
Address:Jl.Dahlia batangluku kab.gowa
Occupation: Housewife
Registered: August, 1st 2014
HISTORY TAKING
Chief complaint:
Erotion and scale in whole body
Anamnesis:
Patient is admitted to RS Wahidin in referral
from RSUD Syeikh Yusuf with complaint of
generalised erotion and scale of whole body with
oral lesion, red eyes and tearing as well as
genital laceration since 1.5 month ago. Initially,
the patient consumed cefadroxyl three days
before skin manifestation.

The cefadroxyl was given as a treatment of
the patients ear due to infection with
effusion. There is also complaint of
difficulty in swallowing food and difficulty
in defecation and urination for more than
one week.
The patient is also consulted to other
department which are Internal Medicine
department, Opthamology department and
ENT department.

TREATMENT
Dexamethasone amp 15 mg/8 hrs/IV
Kenalog Ora Base
Lanolin 10% + Vaselin 30g
Biocream - Face
IVFD : RL/NaCl/Dextrose 5% =
1:1:1


Dermatovenerology status
Location : Generalised
Efflorescence : scale (+), erosion
(+), crust (+), madidans

Additional examination:
- Complete Blood Count (CBC)
August 1st, 2014
WBC : 16,3 10
3
uL
HB :10,71 g/dL
August 4th, 2014
WBC: 19,29 10
3
uL
HB: 9,1 g/dL

PRESENT STATUS
General condition : severe illness
Consciousness : composmentis
Vital sign :
BP : 180/70 mmHg
Pulse : 100x/minute
RR : 32x/i
Temperature : 38,4 C
First photo during admission
Sixth day of treatment

Diagnosis and Treatment from
Internal Medicine Department
Subdivision : Tropical
Infection
Subdivision : GEH
A/ Sepsis ec SSTI A/ GEH
P/ - O2 3 Ltr via nasal canul
- Ceftriaxone 2 g/24 hrs/drips
in 100 cc NaCl 0.9%
- Sistenol 3x1

P/ - Diet hepar
- HP Pro 1-1-1
- Vip Albumin 3x1
- Urdahex 250 mg 0-1-1
Additional Exam::
SGOT : 32
SGPT : 94
Ureum : 213
Keratin : 1,07
Diagnosis and Treatment from
Opthamology department
A/ ectropion ODS
Tx/ - C. Lyters ODS 6x1 gtt ODS
- C. Polygran 3x1 gtt ODS
A/ - Acute Tonsillopharyngitis
- Otitis Media Externa Profunda
Difus dextra et sinistra

P/ - Oral betadine
- Burowi Tampon


Diagnosis and Treatment from ENT
Department
Toxic Epidermal Necrolysis (TEN)
DEFINITION
Toxic Epidermal Necrolysis (TEN) is an
acute life-threatening mucocutaneus
reaction which characterized by mucous
membrane erosion, necrolysis, and
extensive epidermal detachment.
The difference between SJS and TEN
is the involvement of body surface
area (BSA)

< 10% BSA : SJS
10-30% BSA : overlapping SJS-TEN
> 30% : TEN
EPIDEMIOLOGY
SJS and TEN are very rare cases.
Cases incidence of SJS :
1-6 cases per million persons-years
Cases incidence of TEN :
0,4-1,2 cases per million persons-years
Occurs at any ages
Increasing risk : age after the 4
th
decade
Women > Men
ETIOLOGY
Primary cause : DRUGS
High risk drugs : sulfonamide antibacterial,
aromatic anticonvulsant, allopurinol, oxicam
NSAID, lamotrigine, and nevirapine.
Other etiologies : Mycoplasma pneuomoniae
infection, vaccination, graft-versus-host
disease, and radiation.
PATHOGENESIS
I mmunologic pattern of early lesion :
cell-mediated cytotoxic reaction against
keratinocytes massive apoptosis
presence of CD8 T-killer lymphocytes in
dermis and epidermis
CD8 T-killer lymphocyte express - T-cell
receptors and are able to kill through perforin
and ganzyme B
presence of other cytokines, like IL-6, TNF-,
and Fas-L

I mmunologic pattern of late lesion :
>>> monocytes

CLINICAL MANIFESTATION
Prodromal symptoms (1-14 days):
fever, sore throat, chills, headache, malaise

Mucocutaneous lesions :
macule that develope into papules, vesicles,
bullae, urticarial plaques, or confluent
erythema
targetoid lesions
MUCOSAL LESIONS
Mouth :
hemorrhage and
crust on lips
erosions in mouth
covered by necrotic
white
pseudomembrane
ulcerative
stomatitis
Genital :
painful erosions


Eyes :
erosive
conjunctivitis
corneal ulcer
DIAGNOSIS
1. History Taking
- Non-specific symptom (1-3 days) : fever,
stinging eyes, pain upon swallowing
headache, rhinitis, myalgia.
- Epidermal necrolysis : 8 weeks after drugs
consumption
- Initial lesion : erythema macula (body
trunks, then spread to other body sites)
2. Physical Examination
Skin :
- irregular-shaped erythema,
hyperpigmentations, purpuric macules
- targetoid lesion
- confluent necrotic lesions
- Nikolsky sign (+)
- lesions evolve into flaccid blisters
Mucous membrane:
- buccal, ocular, genital mucous
- painful erythema and erosion
- impaired function (impaired alimentation,
photophobia, conjunctival synechiae,
dysuria)




3. Laboratorium Examination
Blood Examination
- anemia - lymphopenia
-leukocytosis - mild thrombocytopenia
- eosinophilia - neutropenia
- blood urea nitrogen - serum urea
- electrolyte imbalance - serum bicarbonate
- hypoalbuminemia - hypoproteinemia

Immunologic Examination
- perifer CD4 lymphopenia

4. Dermatopathology Examination
- sparse apoptotic keratinocytes (suprabasal
layers) which rapidly evolve to a full-
thickness EN and sub-epidermal detachment
- mononuclear cell infiltrate

- among T cells : lymphocytes CD8 with
phenotypic features of cytotoxic cells
- eosinophils : less common in patients with
the most severe form of TEN.
SCORTEN
(Score of Toxic Epidermal Necrolysis)
Fitzpatricks Dermatology in General Medicine 8th Edition
THERAPY
Early recognition
Prompt withdrawal of the offending drugs

1. Symptomatic Treatment
Only patients with limited skin involvement and
SCORTEN score of 0 or 1.
Manage replacement of IV fluid and electrolytes
Nutrition support (nasogastric tube)

Environmental temperature : 28C 30C
Eyes : - examined daily by ophtalmologist
- artificial tears, antibiotic or antiseptic
eyedrops, vitamin A, mechanical
disruption of early synechiae
Pruritus : antihistamine
2. Specific Treatment
High-dosed IV IG : 0,75 mg/kg/day for 4
consecutive days
Cyclosporin : 3 mg/kg/day
Plasmapharesis or Hemodialysis : prompt the
removal of the offending drugs, its
metabolites, or inflammatory mediators.
Corticosteroid :
- controversial
- given in early phase
- dexamethasone 40 mg/day
Cyclosporine A
- powerful immunosuppressive agent
- activates of T helper 2 cytokines, inhibition
of CD8+ cytotoxic mechanisms, and
antiapoptotic effect
DIFFERENTIAL
DIAGNOSES
SJS TEN
ERYTHEMA
MULTIFORM
S.S.S.S
PEMPHIGUS
VULGARIS
Etiology
drugs, infection,
vaccination, graft-
versus-host disease,
radiation
drugs, infection,
vaccination, graft-
versus-host
disease, radiation
autoimmune,
drugs, viral or
bacterial infection
Staphylococcus
aureus
genetic,
autoimmune
Clinical
Features
macule, papule,
plaque, erosion,
ulcer, necrosis,
blistering,
conjunctivitis
macule, papule,
plaque, erosion,
ulcer, necrosis,
blistering,
conjunctivitis,
epydermolysis,
Nickolsky sign (+)
in erithematous
area
erythematous
macule, well-
marginated,
edematous papule,
iris lesion
desquamation,
skin exfoliative in
sheets, lesions
more superficial
(granular layer),
Nickolsky sign
(+)
mucous erosion
and thin-walled,
flaccid, easily
rupture bullae,
Nickolsky sign
(+)
Predilection trunk, palms, soles trunk, palms, soles
dorsal hands

symmetrically,
acrally
neck, groin,
axillae
mouth, burn or
skin injury sites
Age
increasing risk at
age > 40 y.o

woman > man
increasing risk at
age > 40 y.o

woman > man
adolescence
neonates &
children
middle age

woman = man
COMPLICATIONS
Acute phase : sepsis is the most common
complication
Respiratory system : bronchopneumonia
Genitourinary system : acute tubular necrosis,
renal failure, penile scar, or vaginal stenosis
Gastroenterology system : esophageal stricture
Ocular system : corneal ulcer, anterior uveitis,
panophtalmitis, blindness
PROGNOSIS
SJS TEN : life-threatening diseases
Overall hospital mortality of TEN is 22-25%
Mortality rate of SJS : 5-12%
Mortality rate of TEN : >30%