Stevens-Johnson Syndrome

IDENTITAS PASIEN• Name : Mr R• Gender : Male• Age : 34 y.o.• Marital Status : Married• Religion : Islam• Address : Ternate• Ethnicity : Ambon• Nationality : Indonesia• Occupation : Entrepreneur

ANAMNESIS

Chief Complaint: Wound in the lips, eyes and penis

Chief Anamnesis: First experienced ± 2 weeks ago, at first there were only a red spot appeared around hands and body and then a blister appear on lips and eyes

• Patient treated in RSUD Ternate with high fever, and treated with paracetamol and injection (unknown). 3 days later a red spot appeared and diagnosed morbili by doctor. Doctor continued paracetamol treatment and 9 days later, blister were present in lips,eyes, and penis which were accompanied by swelling and pus, the whole body was blackened as well

• Patient feels no improvement while treated in the hospital. So, the patient ask to leave hospital to find another doctor. There, he was diagnosed with Stevens Johnson Syndrome and referred to Wahidin Sudirohusodo Hospital

• History of drug allergy (-)• History of previous illness:

Patient is currently on MDT for 2 months

General Status

• General Condition : Severely ill• Consciousness : Compos mentis• Nutritional Status : Deficient• Hygiene : Deficient• Vital Sign :

- Blood Pressure : 130/70 mmHg - Pulse : 96 x/minute- Breathing : 22x/minute- Temperature : 38,2°C

Physical Examination

• Head : Anemia (+/+), Ikterus (-/-), • Heart/Pulmonary : normal• Abdomen : Ascites (-)• Extremity : Edema lower extremity (-)• Lymph Node : No Enlargement

Dermatology Status

• Locationregio orbitalis D/S , regio labialis, dan regio genitalis

• EffloresenceOrbita : Ulkus, hemorrhagic crustLabia : Erotion, excoriation, hemorrhagic crustGenital : Ulkus, madidans with pus

Results of laboratory tests

• RBC : 0,99x106/mm3 ↓ • HGB : 4,3 gr/dl ↓• HCT : 11,3 % ↓• MCV : 113 um ↑• MCH : 42,8 pg ↑• MCHC : 37,5 gr/dl ↑• PLT : 53 x 103 / ml3 ↑

• GDS : 119 mg/dl• Ureum : 72 mg/dl ↑• Creatinin : 1,50 mg/dl ↑• Bilirubin total : 13,73 mg/dl ↑• SGOT : 47 u/l ↑• SGPT : 38 u/l • Albumin : 2 g/dl ↓

DIAGNOSIS

Swollen eye and blister

DIAGNOSIS

Blackened skin and peeled

DIAGNOSIS

Wound in penis and pus

Terapi

ResumeMan 34 y.o. comes with a wound in eyes,

lips and penis Patient treated in hospital with high fever,

and treated with paracetamol and injection (unknown). 3 days later a red spot appeared, 9 days later, blister were present in lips, eyes, and penis which were accompanied by swelling and pus, the whole body was blackened as well

Patient feels no improvement while treated in the hospital. So, the patient ask to leave hospital to find another doctor. There, he was diagnosed with Stevens Johnson Syndrome and referred to Wahidin Sudirohusodo Hospital

General Status: patient is severely ill, compos mentis, nutritional status deficient, hygiene deficient.Vital Sign: blood pressure 130/70 mmHg, pulse 96 x/minute, breathing 22 x/minute, axilla temperature 38,2°C. physical examination reveals reranemia (+/+)

On skin examination, at orbita obtained effloresensi ulcers and hemorrhagic crusts, the labia obtained erosion and hemorrhagic crust, and ulceration of the penis and madidans with pus.

Thank you

DISCUSSION

DEFINITION

Stevens-Johnson syndrome is a collection of clinical symptoms of mucocutaneous eruption characterized by a triad of disorders of the skin, mucous orifice, and the eye accompanied by severe general symptoms.

ETIOLOGY

Hypersensitivity to infection virus bacterium mycobacteria Mycoplasma pneumoniae protozoa fungi

ETIOLOGY

• Immunization / hyposensitization Diphtheria, pertussis, polio, typhoid, and measles

• hyposensitization pollen, poison ivy • Sensitivity to food, drugs, neoplasms,

connective tissue disease (SLE).

EPIDEMIOLOGY

• Incidence 1,2 to 6 per million person-years.• Common in adults > 40 years old• Risk factor for HLA-B12, SLA, and HIV disease

PATHOGENESIS

• Pathogenesis of SJS is only partially understood. • It is viewed as a cytotoxic immune reaction aimed

at the destruction of keratinocytes expressing foreign (drug-related) antigens. Epidermal injury is based on the induction of apoptosis.

• The nature of the antigens that drive the cytotoxic cellular immune reaction is not well understood.

THERAPY

• Gentamicin injection 80 mg iv/12 Hours (1-1,5 mg/kgBB/).

• Metronidazole infusion 0-0-1/24 hours/iv.• Improvements fluid balance, electrolyte, and protein.• Cleaning debris only in the necrotic skin.• Treating concomitant infections, including sepsis.

THERAPY

Identifying and stopping the drug causes Care and treatment of eye disorders Special care in place to prevent infections

DIFFERENTIAL DIAGNOSIS

Eritem Multiformis

Toxic Epidermal Necrolysis

PROGNOSIS

• Prognosis is good if regular treatment.Recurrence can occur if patients do not discontinue the drug that triggered.