A 3-year-old male patient, with no previous history of importance, who began his illness 15 days before admission to hospital when he presented with a tonic-clonic seizure with deviation of the gaze and lateralised movements to the left lasting about 20 minutes, unrelated to hyperthermia. Eight days after this first episode, he presented again with a seizure of the same characteristics, this time with hyperthermia, poor general condition and sporadic non-productive cough. She started treatment with Amoxicillin, Paracetamol, Diclofenac and Diphenylhydantoin.
He attended a scheduled appointment for an electroencephalogram, but was referred to the emergency department for a fever peak of 40º C. He also had hyporexia, hypotension, hypotension and a high blood pressure. Hyporexia, poor general condition, reddened labial mucosa, hyperemic conjunctivae, painful herpes-like oral lesions, with abundant sialorrhoea. Cervical adenomegaly on the left side measuring 1.5 cm, soft abdomen and intact skin were palpated. A probable diagnosis of Kawasaki syndrome was made on admission.
In the first 24 hours of post-admission evolution he presented clinical deterioration with toxic appearance, oedema of the mouth and lips, purulent discharge from both conjunctivae, tongue with whitish plaque, exanthematous rash in cephalo-caudal direction, maculopapular, non-pruritic, with positive Nikolski's sign on the face.

Forty-eight hours after admission, confluent vesicles and their sphacelation appeared on the anterior and posterior thorax, cheeks and auricular pavilions, bleeding lips, facial oedema with the presence of a whitish layer on the tongue and pharynx with greenish retro-nasal discharge, he was admitted to the Paediatric Intensive Care Unit because of the possibility of laryngeal oedema, and was diagnosed with respiratory distress characterised by intercostal and subcostal stretching, nasal flaring and an oximetry saturation of 85%.

On the third and fourth days of hospitalisation, significant sloughing of the epidermis was observed, mainly on the face, loss of eyelashes, sloughing of the mucous membranes of the cheeks and dorso-lingual, anterior and posterior thorax, upper abdomen, upper arms and penis, with an estimated 45% of the body surface area affected. Water and electrolyte requirements were increased using Galveston's formula and medical treatment was started with Clindamycin, Amikacin and Methylprednisolone. Difehylhydantoin and NSAIDs were discontinued in view of the possible diagnosis of Staphylococcal scalded skin syndrome versus Stevens Johnson syndrome.

Laboratory tests showed:
-BH Hgb: 12.4, Ht. 39.1%, VCM 80.2, HCM 25.5, CMHG 31.7, Leukocytes 19.6, Lymphocytes 29%, Eosinophils 3 %, Segmented 66 %, Bands 1 %, Monocytes 1 %, Platelets 188,000, ESR 6mm/h.
-EGO: Density 1.020, pH 5.0, Yellow colour, Leukocytosis 2-3/field, Trace ketones, Scanty crystals and scanty epithelial cells. Rest negative.
-Electrolytes: Na 139, K 4.2, Cl 101.
-Blood chemistry: total bilirubins 15, unconjugated bilirubins 29, total proteins 7.1, albumin 4.0, globulins 3.2, alkaline phosphatase 255, GGT 22, ALT 49, AST 65, glycaemia 148 and creatinine 0.55.
Surgical lavage and skin biopsy was performed in the right inguinal region and a 3-way central catheter was placed in the left groin to initiate total parenteral nutrition. The bloody areas extended to 60% of the CS as well as to the oral and perianal mucosa, with easy bleeding, which were covered with Lassar's paste and Polymyxin. On both corneas a fibrous cream was visible due to corneal sphacelation, which was easily removed and local medication based on Moxifloxacin eye drops, Ciprofloxacin ointment and ocular lubricant was applied.

The entire systemic antibiotic regimen was changed to Ceftriaxone, Vancomycin and Fluconazole, in addition to analgesia with Midazolam, Nalbuphine, Diazapam, Polymyxin lip drops and Gamma globulin (total dose of 12.8 g).
Skin biopsy confirmed the structural alterations as a severe form of Erythema multiforme of the Toxic Epidermal Necrolysis type probably secondary to drugs (Anticonvulsants). Blood culture and cultures of pharyngeal and ocular secretions were negative.
Further serial surgical washings were performed under sedation and the wounds were covered with hydrocolloid dressings. There was no improvement of the lesions until the third week. Both eyelids showed synechiae that were released multiple times, with corneal re-epithelialisation. She progressed towards improvement and was transferred from the intensive care unit to the hospital ward.
Once the abdomen had healed, a gastrostomy was performed given the persistence of intraoral lesions. The central catheter and Foley tube were removed due to persistent fever. Catheter tip cultures revealed Staphylococcus aureus infection on the 30th day of hospital stay. He was discharged 36 days after admission in good general condition and continued medical treatment with psychological support in outpatient clinics.
He currently has sequelae due to skin dyschromia, photophobia, he is starting to walk with assistance, as well as tolerance to oral food. She continues to be monitored by ophthalmology.

