steven - johnson syndrome

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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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Second level

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Second level

Third level

Fourth level

Fifth level

12/03/2015



Second level

Third level

Fourth level

Fifth level

12/03/2015

steven - johnson syndrome

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