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.
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.
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
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 .