Case presentation Steven-johnson syndrome.pptx

case presentation of steven johnson syndrome.
A 12-year-old female child received in ER with complaints of
Rashes all over the body for one day
Vomiting for one day
Swelling over face for one day
Fever since night.
how to approach a case of steven johnson syndrome, detailed Hx, examination followed by investigations.

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

10. Differetial diagnosis SJ syndrome TEN syndrome Staphylococal skin scalded syndrome Toxic shock syndrome

11. 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.

12. 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

13. 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

14. etiology Allopurinol Carbamazepine Sulfonamides Phenobarbital Phenytoin Valporic acid NSAID (MELOXICAM) Sertraline Abacavir

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

16. 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 .

17. Thank you