AHMAD SAIFUL ASYRAF ANDI INAYAH AZIS

C 111 08 767 C 111 08 292

Patients Identity
Name
Gender Age

Marital Status
Address

Admission Date

: Muhaenah : Female : 57 years old : Married : Tamalanrea : 11 April 2012

History Taking
Chief complaint : Red spots all over the body. Happened since 2 days ago after consuming bodrex, paracetamol and unknown antibiotics. Brief anamnesis : It started with red spots only at the back and then spread all of the body. Her lip was swollen at first. Then it became an ulcer. Both of her eyes was red Itchy (-), Fever (+) before red spots presented Previous Disease History : Coronary heart disease and Hypertension

Physical Examination
Anemic (-), icterus (-), cyanoses (-) Diffuse erythema covering > 90% of the

body surface area, redness on both eyes and wound on the upper lip Cor/ Pulmonal : Normal Abdomen : Normal, peristaltic (+)

Current Status
General status
Composmentis
Moderate Sickness Adequate nutrition Moderate Hygiene

Vital sign (in normal limit)

Blood pressure : 130/90 mmHg Pulse rate

: 96 x/min Respiration rate : 24 x/min Temperature : 40.2 C

Dermato-venerology Status
Location 1 : Regio Generalized Effloresensi : Erythema papule, Squamatous, Ekskoriation
Location 2 : Regio Oral Effloresensi : Erosion, Crusta, hyperpigmentation Location 2 : Regio Facialis Effloresensi : Conjuctivitis

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Laboratory Result
Ureum Creatinin GDS SGOT SGPT Natrium Kalium Chloride

: 20 mg/dl : 1,7 mg/dl : 87 mg/dl : 22 U/l : 47 U/l : 134 mmol/l : 3,9 mmol/l : 109 mmol/l

Recommended Examination
Not specific, can be laboratories examination on

WBC Consult ENT department Consult eye department Consult mouth and teeth department

Resume
A 57 years old woman came to the hospital with complain of red spots all over the body. It happened since 2 days ago after consuming bodrex, paracetamol and unknown antibiotics. It started with red spots only at the back and then spread all of the body. Her lip was swollen at first. Then it became an ulcer. Both of her eyes was red Itchy (-), Fever (+) before red spots presented Previous Disease History : Coronary heart disease and Hypertension

Diagnosis
Steven Johnson Syndrome

Treatment
1. IVFD RL : NaCl : dextrose = 1:1:1 20

drips/min 2. Dexamethasone inj. 1 amp/ 4 h./ iv 3. Gentamicin 1 amp/12 h./ iv 4. Kenolog IU oral base

STEVEN JOHNSON SYNDROME

Stevens-Johnson syndrome (SJS) is acute life-threatening mucocutaneous reactions characterized by extensive necrosis and detachment of the epidermis.

The etiology of Steven Johnson Syndrome is still unclear; however, it is now established that drugs allergy are the most important etiologic factors. More than 100 different drugs have been reported as possible causes

Pathogenesis
Even if the precise sequence of

molecular and cellular events is incompletely understood, several studies provide important clues to the pathogenesis of SJS with related with immune system and drug reactions.

Intake of some drugs causes the abnormal reaction of immune system. Usually hypersensitivity reaction type II

The immunologic pattem of early lesions suggests a cell-mediated cytotoxic reaction against keratinocytes leading to massive apoptosis.

Immunopathologic studies have demonstrated the presence of CD8+T killer lymphocytes in the epidermis and dermis.

Increasing of CD 4, CD 8 and IL -5 and other cytokine causing destruction of keratinocyte.

1.

Cutaneus Lession

The eruption is initially symmetrically distributed on the face, the upper trunk, and the proximal extremities. The distal portions of the arms as well as the legs are relatively spared, but the rash can rapidly extend to the rest of the body within a few days and even within a few hours. The initial skin lesions are characterized by erythematous, dusky red, purpuric macules, irregularly shaped.

2. Mucous Membrane Involvement.

Mucous membrane involvement (nearly always on at least two sites) is observed in approximately 90 percent of cases and can precede or follow the skin eruption. It begins with erythema followed by painful erosions of the buccal, ocular, and genital mucosa

 This usually leads to impaired alimentation, photophobia, conjunctival synechiae, and

painful micturition. The oral cavity and the vermilion border of the lips are almost invariably affected and feature painful hemorrhagic erosions coated by grayish white pseudomembranes and crusts of the lips Common symptoms : fevers, chills, malaise & pruritus

3. Abnormality of the eyes

80% of the cases showed conjunctivitis occur. It also can be conjunctivitis with purulent, bleeding, cornea ulcer and iridosiklitis.

Diagnosis
Approach to patient with SJS rapid confirmation (examination of frozen biopsy speciman may help) elimination of differential diagnoses (biopsy and direct immunofluorescence testing) evaluation of disease severity

Differential Diagnosis
Toxin Epidermal Necrolisis

Treatment
Because of the importance of immunologic and cytotoxic mechanisms, a large number of immunosuppressive and anti-inflammatory therapies have been cited to halt the progression of the disease

CYClOSPORIN A cyclosporin is a powerful immunosuppressive agent associated with biologic effects that may theoretically be useful in treatment of SJS: activation of T helper 2 cytokines, inhibition of CD8+ cytotoxic mechanisms, and anti-apoptotic effect through inhibition of Fas-L, nuclear factor K B,

HEMODIALYSIS the rationale for using hemodialysis is to prompt the removal of the offending medication, its metabolites, or inflammatory mediators such as cytokines.

1. Dexamethasone inj. 1 amp/ 4 h./ iv or Metylprednisolone inj. 1 amp/ 4 h./ iv 2. Antibiotic (ex : ciprofoxacin 2 x 400mg/IV) 3. Dextrose 5%, NaCl 9%, RL = 1 : 1 : 1

Prognosis
Good if early treatment Poor if brochopneumonia occur and if purpura is

wide and covering all over the body