Current work aims to present a case of an 8-years old girl with a history of chemosis of the conjunctiva with fevers. These symptoms appeared from full health and were followed by maculopapular exanthema the next day, which progressed to a vesicular exanthema. Moreover, enanthema of the oral cavity appeared, together with the elevation of inflammatory markers with thrombocytopenia. The patient's medical history showed that patient underwent adenotomy and an even of bronchitis of streptococcal origin (Streptococcus pneumoniae) within 1 year (2014). Except for the above two conditions, the patient had been healthy, without a record of idiopathic or any known allergies, as well as without a history of prolonged use of medications. Anamnesis did not reveal any history of severe medical conditions in the family. No aetiological association between the development of the disease and the use of any drugs according to the ALDEN criteria was found. The repeated serological examination did not detect any antibodies against infectious agents (herpes simplex virus 1,2, varicella-zoster virus, cytomegalovirus, parvovirus, Mycoplasma pneumoniae, Chlamydia pneumoniae, Legionella pneumophila, HIV 1,2, hepatitis viruses).
After the hospitalization, an exfoliative disease was suspected and corticosteroid therapy initiated immediately. Highly suspicious clinical signs of TEN (Figures 1A,C,D) were confirmed by biopsy (Figure 1B). Despite the corticosteroid and (newly initiated) intravenous immunoglobulin (IVIG) therapy, the disease progressed and the patient was transferred on Day 8 from the Pediatric Intensive Care Unit of the local hospital to the Burn Intensive Care Unit (BICU) of the University Hospital Brno. The BICU is a specialized center with experience and expertise in the management and surgical therapy of TEN patients who opted for this type of treatment. On admission to the BICU, the patient was breathing spontaneously. Sixty per cent of the Total Body Surface Area (TBSA) was exfoliated (face, neck, thorax, upper extremities and proximal parts of lower extremities; Figure 1A). Moreover, a symblepharon of both anterior segments of the eyeball was found. Due to the relatively high damage to the mucosal surfaces of the upper respiratory tract, the patient needed intubation during the introduction to general anesthesia, along with the placement of central venous and permanent urinary catheters. Bronchoscopy revealed a fragile and bleeding mucosa; therefore, the decision was made to keep the patient intubated. After the surgery, a girl was transferred to the Department of Pediatric Anaesthesiology and Resuscitation of the University Hospital Brno (PARU) for further intensive care.
In the PARU the patient was administered intravenous methyl-prednisolone (starting on Day 5, 80-40-40 mg every 8 h), antimicrobial therapy consisting of oxacillin (800 mg every 6 h), cefotaxime (1.5 g every 8 h) and, due to the increase of ss-D-glucan level (155 pg/ml), fluconazole (150 mg every 24 h) was added into the therapy. The IVIG therapy continued (0.2 g/kg/day, 5 days administration/25 g cumulative dose) and was supplemented with cyclosporin (2.5 mg/kg/day divided into 2 doses, 5 days administration). Immunological blood tests showed a significant decrease of CD3+ lymphocytes (in subpopulations of CD4+ and CD8+) but no immunoparalysis was proven (expression of CD14+ at monocytes was around 100%).
Despite the above therapy, a patient has developed delirium, which complicated extubation (microaspiration) and caused a need for a re-intubation (9th day of hospitalization). Considering the above and perspective of long-term mechanical ventilation, surgical tracheostomy was performed (11th day of hospitalization). After the patient's condition improved, mechanical ventilation was discontinued without any problems and she was transferred back to the Paediatric Intensive Care Unit of the local hospital for further alimentation and rehabilitation (19th day of hospitalization).
Additionally to the systemic therapy, the patient received specialized surgical management of the vast skin defects, comprising of the surgical debridement, wound dressing and stimulation of regeneration. We have decided to us a unique combination of synthetic (cosmetically nonprivileged areas) and biological (cosmetically privileged area) dressings in addition to the standard of wound care. For the very first time as a part of TEN therapy, we have used as a biological dressing a novel material - lyophilized amniotic membrane (Amnioderm ). Amnioderm  was used in the facial area. The remaining exfoliated skin defects were covered using a combination of synthetic dressings (Aquacel Ag + Extra , ConvaTec, Princeton, NJ, USA and Mepilex Ag , MolnlyckeHealth Care AB, Gothenburg, Sweden). The bandages were replaced 3 times a week under general anesthesia. After the application of Amnioderm , we observed a rapid re-epithelization within 48 h (Figure 2). All skin defects were healed by re-epithelization within 3 weeks after admission to the BICU. We observed no scar formation in the area of AM application. During the 3 months follow-up period, we observed a total regeneration of the skin with no signs of pigmentation or scaring at the area of Amnioderm  application (Figure 3). The area treated with the synthetic dressings was regenerating slower. Compared to the area where AM was applied, healing was extended by 4-5 days to complete wound closure. During the following period, post-exercise hyperaemia without and signs of hyperpigmentation persisted in the areas treated with synthetic dressings.