INTRODUCTION:

Stevens-Johnson syndrome and toxic epidermal necrolysis were the first unusual cases I saw as a case study in our hospital. Stevens-Johnson syndrome, also known as toxic epidermal necrolysis, is a kind of severe cutaneous reaction (SCAR), which is a type of adverse drug response (ADR).¹

The majority of people with reactive skin disease have minor, self-limiting symptoms. It is generally a side effect of a medicine or an illness. Steven-Johnson syndrome (SJS) is a severe type of erythema multiforme caused by an immune complex-mediated hypersensitivity response.² Some call it erythema multiforme major, and it affects the skin and mucous membranes.³ With the separation of the epidermis and dermis, there is cell death. Significant involvement of the oral, nasal, ocular, vaginal, urethral, GI, and lower respiratory tract mucous membranes is possible.⁴ Necrosis may result from GI and respiratory involvement. The characteristics of SJS may be indistinguishable or overlap with those of the related skin diseases toxic epidermal necrolysis (TEN).⁵ A recent systematic study of SJS/TEN found a mortality rate of 12.95 % overall and 28.20% for TEN patients in India. Denuding skin affects more than 30% of the body's surface area, according to most dermatologists.⁶ A.M. Stevens and F.C Johnson described SJS in the medical literature for the first time in 1992. Dr A. Lyell coined the term toxic epidermal necrolysis in the medical literature in 1956, and it is also known as Lyell syndrome.⁷

Incidence:

Western society’s occurrence rate of SJS is 1-2 per 1,000,000, in general population. Some information said that females are affected more than male. SJS can affect any individual including children also, but the incidence rate is higher in elderly population.²

Patient and observation:

Patient present history:

The female patient 14-year-old who was admitted to AVBRH on date 05/09/2019 with the chief complaint was a fever for 5-6days painful red rashes and itching for 3days, blister all over body since3days and pus discharge from the mouth since from 3days. After undergoing an investigation she was diagnosed with SJS.

Past history:

At the time of admission the history was taken patient was apparently alright 6 days back then she started having fever it was very high grade, and intermittent and relieved on taking medication. The patient also started complaining of itching for 3days which was followed by blisters all over the body. Patients were taken to a private practice where she was given some treatment but there was no relief. The patient was having complaint of three episodes of seizure disorder, 1^st episode was in May 2019 which and it lasts for 15-20 min, aborted on its 2^nd episode was on 26 July 2019lasting for 15-20min, aborted on its own and the 3^rd episode was on 15th August 2019 lasting for 15-20min, aborted on its own and which was treated with valparin and eptoin.

Etiology:

Steven-Johnson syndrome has been linked to a variety of etiological factors. Most frequently, drugs are implicated. The causes are listed as infectious, which can be caused by a virus, a bacterial infection, or a fungal infection. Antibiotics, anticonvulsants, NSAIDs, and antiviral medicines are examples of drug-induced toxicity. Genetic factors i.e., Carriage of specific human leukocyte antigens has been linked to an elevated risk; Idiopathic in 25-50% of patients.

Clinical Findings:

The patients who developed blisters may form in the mucous membrane and also involve the mouth. Only and also appears in the nose and genital area. The patient has pain due to blister and it led to erosions and bleeding. Mucosal lesions inside the lips of the mouth develop extremely painful and cause difficulty in eating. Lesions in the genitourinary tract occurred in the patient, which might result in decreased urine flow and dysuria.⁴

Physical examination:

The patient shows the sign and symptoms of red and purple rashes, blister, macules, vesicle, and swelling of eyelids. On observation it shows persistent fever and flue like symptoms. In a patient the rash first appears on the upper chest, face palms and soles. After a few days these rash spread to the other portion of the body and its itchy and painful.

Figure-1 A rash on the face and neck, as well as widespread blistering lesions with mucosal involvement.

Figure-2 Skin rash over abdomen

Diagnostic Assessment:

On physical examination and patient history and others investigations revels different outcome with clinical and skin biopsy. The patient skin biopsy has sent to the laboratory for the confirmation of the SJS/TEN,X-ray imaging and blood tests.⁵ The CBC reports reveals the normal hemoglobulin level 11.6% gm/dl, Neutrophils, Lymphocytes, Eosinophils, Basophils, TLC Platelets, MCV, MCH, MCHC all have normal values but Monocytes has 1% and HCT 35.5gm%, decrease in values. Sr. Bilirubin, Total protein, Albumin, Total globulin, Sr. Urea and Sr. Potassium has normal values but Sr. Creatinine 4% that is increased in numbers and Sr. Sodium 134 mEq/l decreases in number.

Cultural report results has shown the growth of inducible Amp C producer pseudomonas species.

Patient report of drug sensitivity shows patient is sensitive to piperacillin tazobactam, Imipenem, Piperacillin and Co-trimoxazole drugs and resistant to Amikacin, Ceftazidime and Ciprofloxacin these drugs.

Therapeutic Intervention:

Medical management:

Patient was treated with antibiotics, oral steroids, antacids and local anesthetic, capsule. The following drugs are Cyclosporine 100mg-50 mg for 7 days, syrup. Nucool-o 2tsp (20 min before meal BD) for 7 days, syrup. Betadine gargle 5ml twice a day, syrup. Mucaine gel 10ml for thrice a day, Quin- KT EID, syrup. PCM 12ml as per if necessary, syrup. Gelusil 5ml thrice a day, syrup. Omnacotril 5ml, syrup. Benadryl 5ml, inj. Ceftriaxone 1gm(IV) (50mg/kg/day) for 12 hourly, inj. Leviteraceta 400mg in 10ml NS IV (1mg/kg/dose) for 12 hourly and syrup. Atrax 5ml for night only. With all these drugs the patient maintain the hydration and electrolytes.

Nursing management:

As per the SCORTEN criteria the nursing care was given to maintain the personal hygiene to prevent further infection.

· Identify and stop the offending agent

· IV fluid given

· Corticosteroids given

· Antibiotic given to treat secondary infection

· Topical anesthetics are used in an oral/mouth wash twice a day to reduce discomfort and allow patients to drink water.

Complication:

There are the following complication the patient were present according to the book complication present with patient complication.

Complication of patients compare with book pictures

S.no	Book picture	Patient Picture
1.	Chronic cicatrizing conjunctivitis	Present
2.	Corneal epithelial defects	Present
3.	Corneal stromal ulcers	Present
4.	Endophthalmitis	Present

Follow up and outcomes intervention:

At the time of discharge the patient conditions were satisfactory her blisters are recovered and she was able to eat food itself. Their relatives were informed about the drug therapy and personal hygiene, all prescribe drug should be taken as per the schedule they should come after 10 days for routine follow up to see the disease outcome.

Patient perspective:

Prognosis:

A 14 year female patient tolerate all the medication and well response within seven days to the therapeutic treatment of the hospital which was given as a treatment.

Informed Consent:

Before taking this case, information was given to the patients and their relatives and Informed consent was obtained from patient as well as relatives.

DISCUSSION:

A female patient was admitted with a chief complaint of seizure attacks in past and the treatment history was, the patient treated with anticonvulsant drugs like eptoin and valparin were she had the severe adverse effect of these drugs and developed blisters in the mucous membrane in oral as well as in the nose and the genital area also there was s/s of rashes and macules and vesicle which led to erosion and bleeding. She undergone with some investigation and diagnosed with SJS/TEN and further treatment was started in AVBRH hospital were after 1 month of complete treatment and nursing care she was healing and got discharge with follow-ups.

Steven-Johnson syndrome (SJS) is an immune-complex-mediated hypersensitivity complex that typically involves the skin and the mucous membranes. Steven-Johnsons syndrome is a serious systemic disorder with the potential for severe morbidity and even death. Stevens-Johnson syndrome⁸. A minor form of TEN with less than 10% body surface area (BSA) detachment. The common trigger drugs in the patient were antiepileptic (Valparin and eptoin drugs). Antiepileptic and antibiotic drugs are causing SJS or TEN along with Non steroidsAnti-inflammatory drugs and allopurinol. Most patients are treated symptomatically⁹.

Two individuals were diagnosed with Stevens-Johnson syndrome, four with Stevens-Johnson syndrome/toxic epidermal necrolysis overlap, and five with toxic epidermal necrolysis. The average duration of drug use was 10 days (2–44 days). Two patients have IgM antibodies against the herpes simplex virus.The average recovery time was 38 days. All patients had maculopapular eruptions and oral mucositis. Ten individuals had vesiculae or bullae, epidermal separation, and ocular involvement. All patients received wound care, H1 antihistamine, and methyl prednisolone, while 7 received intravenous immunoglobulin and 1 received cyclosporine. Two of the patients had secondary lesions, but there was no mortality. Anticonvulsants, antibiotics, and Nonsteroidal anti-inflammatory medications are all implicated in the pathogenesis of Stevens-Johnson syndrome and toxic epidermal necrolysis. Anticonvulsants have been linked to serious illness. Patients who receive adequate wound care and immunosuppressive medication can recover.¹⁰

CONCLUSION:

The patient was admitted to hospital with the chief complaint of three episodes of seizure attack in past for which she was treated with drugs like valparin and eptoin because of which the child got an adverse reaction on her body with symptoms such as painful red rashes, blisters, pus discharge from mouth and her condition was very critical. Immediate treatment was started by a health team member now the patient condition was satisfactory. The patient cure and the healing were started and discharged with follow-ups.

ETHICAL CLEARANCE:

Not required.

AUTHOR’S CONTRIBUTION:

All the authors contribute in the work.

ACKNOWLEDGEMENTS:

We owes sincere thanks to nursing faculty member of SRMM College of Nursing for their guidance and encouragement inspire of their busy schedule, and above all we would like to thanks god almighty and our parents being the guiding star in our life.

REFERENCE:

1. Stevens-Johnson syndrome - Symptoms and causes - Mayo Clinic [Internet]. [cited 2021 Nov 25]. Available from: https://www.mayoclinic.org/diseases-conditions/stevens-johnson-syndrome/symptoms-causes/syc-20355936

2. Rixe N, Tavarez MM. Serum Sickness. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 [cited 2021 Nov 25]. Available from: http://www.ncbi.nlm.nih.gov/books/NBK538312/

3. Erythema Multiforme - StatPearls - NCBI Bookshelf [Internet]. [cited 2021 Nov 25]. Available from: https://www.ncbi.nlm.nih.gov/books/NBK470259/

4. Mechanisms of Microbial Infections [Internet]. [cited 2021 Nov 25]. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7158295/

5. Oakley AM, Krishnamurthy K. Stevens Johnson Syndrome. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 [cited 2021 Nov 25]. Available from: http://www.ncbi.nlm.nih.gov/books/NBK459323/

6. Tang C-H, Ramcharran D, Yang C-WW, Chang C-C, Chuang P-Y, Qiu H, et al. A nationwide study of the risk of all-cause, sudden death, and cardiovascular mortality among antipsychotic-treated patients with schizophrenia in Taiwan. Schizophr Res. 2021 Nov; 237:9–19.

7. Purnamawati S, Febriana S, Danarti R, Saefudin T. Topical Treatment for Stevens -Johnson Syndrome and Toxic Epidermal Necrolysis: A Review. Bali Med J. 2016; 5:92–101.

8. Parveen S, Javed MA. Stevens Johnson Syndrome associated with Lamotrigine. Pak J Med Sci. 2013; 29(6):1450–2.

9. Koh M, Tay Y-K. Stevens Johnson syndrome and toxic epidermal necrolysis in Asian children. J Am Acad Dermatol. 2009; 62:54–60.

10. Çekiç Ş, Canıtez Y, Sapan N. Evaluation of the patients diagnosed with Stevens Johnson syndrome and toxic epidermal necrolysis: a single center experience. Turk Arch Pediatr Pediatri Arş. 2016; 51(3): 152–8.

11. Treatment of Oral Fistulas | IntechOpen [Internet]. [cited 2021 Nov 25]. Available from: https://www.intechopen.com/chapters/50217

Received on 20.12.2023 Modified on 03.01.2024

Int. J. Nur. Edu. and Research. 2024; 12(1):67-70.

DOI: 10.52711/2454-2660.2024.00015