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Steven Johnson Syndrome
Name : Mr. U
Age : 29 years old
Address: Dusun Lengkong Luwu
Status: Single
Date of Admitted : 4th March 2015
Identify of Patient
Main Complain
Itchy at whole body
Anamnesis:
Patient feel itchy at whole body.
This sensation has been felt since 1 weeks ago.
Firstly, the itchy, redness, and the lesion appear at face and spread to the whole body.
Now, the whole body becoming dry and the skin start to peel off.
History Taking
History of medicine taking (+),
ARV (since 2 months ago)
Cotrimoxazole (since 2 months ago, uncontinously)
Fluconazole (since 3 days ago)
History of hospitalization(+) with tifoid fever 1 year ago,
History of allergic (-),
History of the same disease (-),
History of DM(-), HT(-),
History of the same disease in family (-)
History Taking
Anemic (-), Icterus (-), Cyanosis (-)
Cor/pulmonal : normal
Peristaltic : (+) normal
Physical examination
General status : Compos mentis, adequate nutrition
General Condition : Moderate
Hygiene: Moderate
Vital Signs :
Blood Pressure: 120/80 mmHg
Pulse : 88x/minute
RR: 18x/minute
Temperature: 36,8oC
Present Status
1. Dermatology Status
Location : Universal regio
Efflorescency: Thick scales, hyperpigmented macula
Location: Regio orbitalis, oralis
Efflorescency : Crust, erotion, secretion at conjungtiva
Dermatovenerology Status
2. Venerology Status
Location: Regio Scrotalis
Efflorescency: Scales, erotion, erythem
Laboratorium
Routine Blood
Urinalisis
Diagnosis
STEVEN JOHNSON SYNDROME
Differential Diagnosis
NET
Therapy
IVFD RL 28 dpm
Injection Dexamethazone 1 amp/6 hours/IV
Eritromycin 500 mg tab/ 8 hours (per oral)
Vitamin Bcom tab / 8 jam (per oral)
Landin 30% + vacelin alb (twice a day, half of upper body in the morning and half of upper body in the evening )
Fuson cream (at lesion, twice a day)
Stevens Johnson syndrome ( SJS) is acute life threatening mucocutaneus reactions characterized by extensive necrosis and detachement of the epidermis
Etiology :
Drugs ( > 50 % )
Infection
Vaccination
Graft versus host disease
Neoplasm
Radiation
Fitzpatricks in general medicine 7th edition, pg 349
Ilmu penyakit kulit dan kelamin FK UI 6th edition, pg 163
Fitzpatricks in general medicine 7th edition, pg 350
Patomechanism
The medication might induce upregulation of FasL by keratinocytes constitutively expressing Fas, leading to a death receptor-mediated apoptotic pathway
The drug might interact with MHC class I-expressing cells and then drug-specific CD8+ cytotoxic T cells accumulate within epidermal blisters, releasing perforin and granzyme B that kill keratinocytes
The drug may also trigger the activation of CD8+ T cells, NK cells and NKT cells to secrete granulysin, with keratinocyte death not requiring cell contact. IVIg contains antibodies against Fas that can block the binding of FasL to Fas
Bolognia Dermatology 3rd edition, pg 327
Recommended Examination
Laboratory values
Histopathology
Fitzpatricks in general medicine 7th edition, pg 352 353
Treatment and Management
Bolognia Dermatology 3rd edition, pg 330
Prognosis
Dubia
Ilmu penyakit kulit dan kelamin FK UI 6th edition, pg 165
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