D2.60 - Stevens-Johnsons syndrome: the contemporary immunopathology and case study

Stevens-Johnson syndrome is an acute, rare, immune-complex-mediated disease involving the skin and the mucous membranes. It is considered a minor form of toxic epidermal necrolysis, with less than 10% of the body surface area evidencing detachment. Various etiologic factors such as infections and drugs have been implicated in the etiology. Physicians must therefore consider Stevens-Johnson syndrome as a potential complication of treatment, especially when the use of medication is questionable. Diagnosis relies mainly on clinical signs together with the histological analysis of a skin biopsy. The differential diagnosis includes drug hypersensitivity reactions, staphylococcal scalded skin syndrome, acute generalized exanthematous pustulosis, and autoimmune bullous dermatoses. Immunosuppressive medications have been used with some success in patients. Our case report underscores the imperative of teamwork, vigilance, and thorough patient examination to prevent any unfortunate oversights.

A 6-month-old boy was hospitalized at M. Iashvili Central Pediatric Clinic in Tbilisi, Georgia. According to the parents, nasal obstruction, rhinorrhea, and restlessness started two days before hospitalization. The pediatrician assessed it as a respiratory infection and prescribed an antiviral spray and nasal drops. On examination in the hospital, there was a diffuse maculopapular rash on the neck and face, Swelling of the soft tissues of the face, large painful erosions, and blistering rash on the face, trunk, limbs, and mucosal surface. He also had Blepharoconjunctivitis. The patient was diagnosed with Stevens-Johnson syndrome. A council was held, where the patient's condition was assessed as critical, and the patient was managed with supportive care. The patient was prescribed dexamethasone, atarax, and sodium chloride. He was consulted by an ophthalmologist and was prescribed Tobrex and Ophtaquix. Despite the treatment, on the third day after hospitalization, the general condition of the patient worsened. Hyperemia and desquamation of the skin expanded and spread to the dorsal surface, the groin area became swollen and hyperemic. The patient had adynamia, vomiting, and widespread skin pain, which is why Infulgan was prescribed. Under active observation, the treatment continued unchanged, and from the seventh day, the patient's condition gradually improved. On the ninth day, the desquamation of the necrotic areas of the patient's skin was completed, and the picture of blepharoconjunctivitis was not expressed. Paraclinical studies showed positive dynamics. Due to the positive dynamics, the patient was discharged with the appropriate prescription on the tenth day.

Our case shows that Stevens-Johnson syndrome is a rare disease, especially, at this age. It remains a significant challenge for pediatricians, as it requires accurate anamnesis collection, rapid and accurate differential diagnosis, adequate management, and active monitoring during treatment