A case report on Stevens Johnson Syndrome

Monica¹, Gayathri Devi M², S J. Soniya mercy Anbu³, R. Ruby⁴

¹Staff Nurse- Isolation Ward, MAA Kauvery, Hospital, Trichy, Tamil Nadu

²Supervisor -PICU, MAA Kauvery Hospital, Trichy, Tamil Nadu

³Nursing Educator, MAA Kauvery Hospital, Trichy, Tamil Nadu

⁴Senior DNS, MAA Kauvery Hospital, Trichy, Tamil Nadu

Case Presentation

A 11-year-old female child was admitted to our hospital, reporting a history of lip swelling, multiple erythematous lesions located on the face, abdomen, trunk, and palms for the past 7 days, fever lasting 7 days, one instance of loose stool, and diminished urine output for 4 days.

Past history

H/o Recurrent aphthous ulcers (+)

Development history

Attained developmental milestones at appropriate age.

Investigation

S. No	Investigation	Results
1	HB	11
2	PCV	36
3	CRP	74.9
4	LFT	Normal
5	Urine complete	Normal
6	Anti-nuclear, Anti-body indirect test	Positive
7	Nuclear compartment	Weak Positive
8	Cytoplasmic compartment	Negative
9	Mitotic compartment	Negative
10	Hbs AG	Negative
11	HCV	Negative
12	Fluid cytology (TZANCK SMEAR)	Suspicious for occasional acantholytic cells.
13	ANCA-weak positive

Fig (1): Skin eruption in SJS

Blood culture: sterile for 24 -42 hr

Skin biopsy: superficially detached epidermis with necrosis, haemorrhage and haemosiderin laden macrophages. Tzanck smear from blisters showed occasional acantholytic cells.

Management

Child received in hospital with the presenting symptoms of erythematous vesicular lesion over abdomen, trunk, palms, external genitalia, lip swelling, high grade fever, loose stools, cough following analgesic intake (Diclofenac). She was initially treated as an outpatient with oral antibiotics, analgesics, and then came to hospital for further management. On Presenting to ER, child was alert, a febrile, and hemodynamically stable. On examination blepharitis, lip swelling with erosions, multiple aphthous ulcers, multiple erythematous flaccid bullae all over body, vulval erythema. Possibility of Stevens Johnson syndrome/Toxic epidermal necrolysis was considered. The child was started on IV fluids, IV Antibiotic (Inj. Augmentin), oral paracetamol. The Child was then shifted to Isolation ward for further care. Dermatologist opinion obtained, steven Johnson syndrome probable due to diclofenac was considered and advised to start on IV steroid and topical antibiotic. Pediatric ophthalmologist was consulted, on examination bilateral blepharitis with clear cornea and conjunctiva, started on topical antibiotics and steroid.

Stomatologist was consulted as child had swallowing difficulty due to oral ulcers and tube feeds, topical steroids were initiated. Rheumatologist opinion obtained, to rule out SLE, advised to do ANA profile and C3 C4 levels, which was found to be negative with normal C3, C4 levels. Pharmacogenetics testing was sent and report was awaited H er cutaneous lesions healed gradually and tolerate oral feeds well. IV steroids were tapered and stopped and there were no further new lesions during hospital stay. The Child was improved symptomatically and discharged with following advice.

Nursing Management

Throughout the hospital stay strictly followed hand hygiene techniques and moments. Personal protective equipments and barrier nursing protocols adhered. CAUTI, CLABSI bundle care followed.

Treatment Given

IV fluids
Augmentin
Dexamethasone
Pantoprazole
cetrizine
paracetamol
Refresh eye drops
Moxicip eye drops
L-Pred eye drops
Sofarmycin cream
White petroleum jelly

Condition at discharge

Child stable, a febrile, hydration adequate, urine output adequate, vitals stable.

Advice on discharge

High calorie diet and adequate hydration
Dermatologist review

Medication

S. No	Drug Name	Frequency	Route
1	Augmentin (625mg)	BD	Oral
2	Pantoprazole (40mg)	OD	Oral
3	Paracetamol (650mg)	TDS	Oral
4	Triamcinolone	TDS	L/A
5	Loteprednol etabonate	Q6H	Eye
6	Carboxymethylcellulose	Q4H	Eyes
7	Moxifloxacin	BD	Eyes
8	Safranin	TDS	L/A
9	White petroleum jelly	TDS	L/A

Advised the parents to report to hospital immediately if baby has excessive dullness, continuous cough, cold, high-grade fever, fast breathing, difficulty in breathing, excessive diarrhea, vomiting, reduced urine output.

Discussion

Stevens – Johnson syndrome (SJS) is a rare, serious disorder of the skin and mucous membranes, often triggered by medications or infections. It’s characterized by painful blistering and skin shedding, resembling a severe burn, and can also affect the eyes, mouth, and other mucous membranes.

Symptoms

One to three days before a rash develops, you may show early signs of Stevens-Johnson syndrome, including:

Fever
A sore mouth and throat
Fatigue
Burning eyes

As the condition develops, other signs and symptoms include:

Unexplained widespread skin pain
A red or purple rash that spreads
Blisters on your skin and the mucous membranes of the mouth, nose, eyes and genitals
Shedding of skin within days after blisters form

Causes

Stevens-Johnson syndrome is a rare and unpredictable illness. Usually, the condition is triggered by medication, an infection or both.

Drugs that can cause Stevens-Johnson syndrome include:

Anti-gout medications, such as allopurinol
Medications to treat seizures and mental illness (anticonvulsants and antipsychotics)
Antibacterial sulfonamides (including sulfasalazine)
Nevirapine (Viramune, Viramune XR)
Analgesics, such as Acetaminophen, Ibuprofen and Naproxen sodium.
Infections that can cause Stevens-Johnson syndrome include pneumonia and HIV, Herpes simplex virus, Mycoplasma pneumonia.

Risk factors

An HIV infection.
A weakened immune system.
Cancer
A history of Stevens-Johnson syndrome.
A family history of Stevens-Johnson syndrome.

Complications

Dehydration
Sepsis
Eye inflammation, dry eye and light sensitivity. In severe cases, it can lead to visual impairment and, rarely, blindness.
Acute respiratory failure
Permanent skin damage: when the skin grows back following Stevens-Johnson syndrome, it may have bumps and unusual coloring (dyspigmentation), scars, hair fall, diminished growth of finger and toenails.

Diagnosis

Diagnosis is mainly clinical but may include

Skin biopsy
Blood test
Cultures to rule out infection

Prevention

Avoid known trigger drugs
Genetic testing (HLA testing)
Use medications cautiously
Promptly treat infections
Patient education and medical alert
Vaccinations and immune health.

A case report on Stevens Johnson Syndrome

A case report on Stevens Johnson Syndrome

Monica1, Gayathri Devi M2, S J. Soniya mercy Anbu3, R. Ruby4

Nightingale Journals

Monica¹, Gayathri Devi M², S J. Soniya mercy Anbu³, R. Ruby⁴