A 12-year-old female presented with rashes all over her body, vomiting, and facial swelling for one day with a fever since the night before. Her father reported that she had developed itching, rash starting on her lips then spreading to her face and body along with vomiting, eye redness, tearing and discharge. She had been taking carbamazepine for one week. A differential diagnosis of Steven-Johnson syndrome, toxic epidermal necrolysis, staphylococcal scalded skin syndrome, or toxic shock syndrome was made. Management involved stopping the offending drugs, supportive care, and treatment to prevent secondary infections.

1. CASE PRESENTATION
by Dr Asad

2. PRESENTING COMPLAIN
 12 year old female child received in ER with
complain of
 Rashes all over the body for one day
 Vomiting for one day
 Swelling over face for one day
 Fever since night

3. HOPC
 According to the father his child was alright then yesterday pt
developed itching & rash started from lips then progress to the
face and over the body associated with vomiting , multiple
episodes, non-projectile, white colour. Patient having fever since
night, high grade fever, undocumented, without chills and rigors
relieved with paracetamol, also associated with redness of eyes,
lacrimation and pus discharged from eyes. drooling of saliva and
oral ulcers. According to the father Patient was taking medicine
carbamezapine for some reason from 1 week.
 Systemic history
 CNS = no Hx of fits and ALOC
 CVS= normal
 Respiratory sys= difficulty in breathing
 GI= nausea & vomiting
 Genitourinary= normal

4. PAST MEDICAL HISTORY
 Father gave wage history of 2 month back patient
suddenly got weakness of right side of the body for
which they treated as a OPD case
 CT-scan brain was done for weakness but there
was no abnormality was identified
 No past surgical HX
 No blood transfusion hx
 No recent vaccination hx

5. BIRTH HISTORY
 Full term NVD at hospital
 No hx of birth asphyxia or meconium aspiration.
 Mother was anemic during pregnancy for which
doctor prescribed iron and folic acid supplements.
 Mother had no fever, HTN ,DM or other illness
during pregnancy.

6.  Drug history
 Patient is taking anti-emetics, paracetamol,
carbamazepine and anti-allergic from 1 weeks
 Family history
There is no any chronic illness in family
3 siblings , all healthy and alive.
No history of fits and measles in the family.
No history of blood transfusion in the family.
Socioeconomic Hx
Father is laborer
Socioeconomic status is poor.
One room is being shared by 6 family members.
No proper hygiene.
Line water without boiling.

7. DEVELOPMENTAL HISTORY
 Neck holding = 3-4 months
 Sitting = 5-6months
 Walking=15 months
 Intellectually patient is normal
 Two month back patient developed weakness of right
side of the body which leads to loss of ability to walk but
after 2-3weeks pt regain ability to walk but limping
persist.

8. SYSTEMIC EXAMINATION
 CNS= conscious
 CVS= S1+S2 audible
 Chest= b/l air entry with marked conducting sounds
 ABD= soft, NVM and bowel sounds audible
 Vitals
 HR= 144b/min
 RR= 30b/min
 Temp= 100f
 CRT <3sec
 SpO2= 94%

9. LAB INVESTIGATIONS

12. DIFFERETIAL DIAGNOSIS
 SJ syndrome
 TEN syndrome
 Staphylococal skin scalded syndrome
 Toxic shock syndrome

13. STEVEN-JOHNSON SYNDROME
 Steven Johnson syndrome is an immune complex
mediated hypersensitivity characterized by the skin
and mucous membrane involvement. Extensive
widespread necrosis, causing epidermis to
separate from the dermis.
 Classification
 Steven johnson syndrome; minor form of toxic
epidermal necrolysis, with less than 10% body
surface area involvement.
 Overlapping SJS/TEN : 10-30% BSA
 TEN: involvement more than 30%of the BSA.

14. CLINICAL FINDINGS
 Cutaneous lesions: Erythematous macules develop
into central necrosis to form vesicles, bullae and
areas of denudation on the face, trunk and
extremities. Skin tenderness is minimal.
Involvement of 2 or more mucosal surfaces .
 Fever
 Malaise
 Myalgia and arthralgias
 Nausea & vomiting
 Burning sensation in the eyes
 Cough

15.  Corneal ulceration, anterior uveitis,
panophthalmitis.
 Bronchitis and pneumonitis.
 Myocarditis
 Hepatitis
 Enterocolitis
 Polyarthritis
 Acute tubular necrosis may lead to renal failure
 Strictures
 Insensible water loss
 sepsis

16. ETIOLOGY
 Allopurinol
 Carbamazepine
 Sulfonamides
 Phenobarbital
 Phenytoin
 Valporic acid
 NSAID (MELOXICAM)
 Sertraline
 Abacavir

17. INVESTIGATIONS
 Nonspecific laboratory abnormalities which includes
 1/ leukocytosis
 2/ increased ESR
 3/ occasionally increased liver transaminase level
 4/ decreased serum albumin level

18. MANAGEMENT
 Management of SJS is supportive and symptomatic.
 Stop the offender drugs ASAP
 Ophthalmologic consultation to prevent the ocular sequelae
 Oral lesions should be managed with mouthwashes and
glycerin swab.
 Vaginal lesions should be observed and treated to prevent
strictures and fusion.
 Topical anesthetics
 Denuded skin lesions can be cleaned with saline.
 Antibiotic therapy to prevent secondary infections.
 I/V fluids and nutritional support
 IV immunoglobulins .

19. Thank you