Stevens-Johnson Syndrome: A Case Report on Acute Hypersensitivity Reaction and Management

Abstract

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe, life-threatening conditions characterized by extensive epidermal necrosis and mucosal involvement. SJS is defined by less than 10% epidermal detachment, while TEN is associated with more than 30?tachment, and SJS-TEN overlap occurs between 10-30%. We present a case of an 11-year-old female with a diagnosis of SJS-TEN overlap syndrome. The patient presented with extensive rashes, headache, watery stools, and hematuria. The rashes initially appeared on her chest and spread to involve the face, limbs, and abdomen, with the development of black scabs that bled upon removal. Laboratory findings revealed leucocytosis, elevated liver enzymes, and increased C-reactive protein. Due to the acute nature of the symptoms, hematological investigations were prioritized. Based on clinical and laboratory findings, the diagnosis of SJS-TEN overlap was made. The patient was treated with intravenous Vancomycin, oral Azithromycin, Meropenem, Lipomadol, Lidocaine, Fusidic acid, and Ciprofloxacin ointment. Daily reviews showed significant healing of the lesions, with complete recovery of all lesions observed after approximately 50 days. Early diagnosis, appropriate medication, and expert care contributed to a positive outcome for the patient.

Keywords

Introduction

Stevens-Johnson syndrome (SJS), an extremely severe acute hypersensitivity reaction, causes extensive necrosis on the skin and the mucous membrane. The normally accepted mechanism of SJS is a cytotoxic immune reaction in keratinocytes that leads to widespread keratinocyte apoptosis. With less than 10% epidermal surface invasion, the disease is called SJS, and with more than 30%, it is called toxic epidermal necrolysis (TEN) (1,2). The incidence of SJS and Toxic Epidermal Necrolysis (TEN) is 1.0–6.0 per million and 0.4 to 1.2 million respectively, nevertheless, this is twofold for the Asian people (3). SJS and TEN are in the same spectrum of diseases but affect different total body surface area percentages: SJS, < 10%; SJS–TEN overlap, 10–30%; and TEN, > 30%. The overall incidence of SJS–TEN is unclear, although a US study indicated that the incidence could be between 1.58 and 2.26 per million people annually, and the mortality rates could be as high as 4.8–14.8%. Even after recovery, blindness can be a serious sequela in some patients. Thus, prompt diagnosis and appropriate treatment are necessary. (4,5)

Case Report

An eleven-year-old female patient brought to the pediatric department with the complaints of extensive rashes covering her entire body, headache, watery stools for the past 4 days, and blood in her urine for one day. The patient's current condition showed that her chest received the first erythematous macular lesions which spread toward her face and involved all body surfaces. The skin condition now extends across her face together with her chest and back and abdomen and upper and lower limbs. The skin lesions now present black scabs which bleed after the scabs are taken off. The patient experienced a sudden onset of fever four days ago that kept intensifying but did not include chills or rigors. The patient sought medical advice from an unlicensed practitioner who caused rashes with burning pain to develop on her mouth and chest region. Laboratory investigations showed leucocytosis (white blood cells are 11500 micro liters, SGOT (60 U/L), SGPT (86 U/L) and raised C-reactive protein 24.0 g/ml. We decided to focus only on hematological investigations for the patient due to the acute nature of the lesions, as the patient was experiencing significant discomfort. Based on the patient's medical history, clinical examination, and physical findings, our diagnosis was SJS–TEN overlap syndrome. The patient was treated under the expert care of a pediatrician with intravenous Vancomycin (4 ml in 60 ml normal saline, given four times a day) for 10 days. Afterward, the treatment was switched to oral Azithromycin (250 mg once daily) for the next 5 days. Additionally, Inj. Meropenem (9 ml, 12 ml), Inj. Lipomadol, Lidocaine, and Fusidic acid were applied to the lips and skin lesions three times a day. Saline gargles were prescribed thrice daily for the oral cavity ulcers, and Ciprofloxacin ointment was used to treat ocular infections. The patient was reviewed daily, and significant healing was observed in the oral cavity and skin lesions. After 15 days, the patient was reassessed, and the lesions on all areas had completely healed after approximately 50 days.

DISCUSSION

Stevens-Johnson Syndrome is a rare, life-threatening disorder characterized by mucosal erosions, widespread skin detachment, and systemic involvement, often triggered by medications, infections, or systemic diseases (6). This condition begins with flu-like symptoms such as fever, malaise, and sore throat, followed by the appearance of erythematous macules that evolve into vesicles and necrotic lesions (7). Drugs such as, sulfonamides, allopurinol, anticonvulsants and NSAIDs are most commonly implicated as triggers of SJS, with the most severe cases progressing to toxic epidermal necrolysis (TEN), a condition where more than 30% of the body's skin detaches (8). The pathophysiology of SJS involves a T-cell mediated immune response resulting in keratinocyte apoptosis and widespread epidermal necrosis (9). Diagnosis is primarily clinical, supported by skin biopsy and laboratory tests. Management of SJS typically involves discontinuation of the offending drug, fluid resuscitation, wound care, pain management, and in severe cases, the use of immunosuppressive agents (10). Early intervention and careful monitoring are critical to reducing morbidity and mortality, as complications such as sepsis, multi-organ failure, and blindness can occur (11). Although outcomes have improved with advances in treatment, long-term follow-up is often necessary due to potential sequelae like scarring and ocular complications (12).

CONCLUSION

In Conclusion early diagnosis and prompt, appropriate treatment led to significant improvement and complete healing of the lesions. The use of systemic antibiotics, topical treatments for skin and mucosal lesions, and supportive care were crucial in managing the patient’s condition. This case highlights the importance of early detection and treatment in preventing long-term complications such as blindness and scarring.

Declaration of patient consent: The authors certify that they have obtained all appropriate patient consent forms.

Financial Support and Sponsorship: NIL

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