Long-term consequences of Lyell's syndrome suffered in childhood

The article provides a review of literature on the long-term consequences of Lyell's syndrome (one of the forms of toxic epidermal necrolysis). Among them: chronic eczema with xerosis and itching, skin depigmentation, hypertrophic and keloid scars, nail lesions, eyes, including dry eye syndrome and chronic conjunctivitis with/without obliteration of the nasolacrimal canal, kidney disease, lung disease, Sjogren's syndrome, Hashimoto's thyroiditis and others. Clinical case. The authors present their clinical case of an 11-year-old girl who suffered from Lyell's syndrome at the age of 8 and has been observed for the past three years, share their clinical and laboratory-instrumental observations and treatment strategies. In particular, they report about the revision of the clinical diagnosis with which the girl was admitted to the OHMADYT hospital, from BA to chronic secondary obstructive bronchitis, the detection of chronic conjunctivitis with obliteration of the nasolacrimal canals, chronic sinusitis, secondary immune deficiency, keloid skin scars. Conclusions. Despite constant rehabilitation therapy and a significant improvement in the general condition of the girl, it was not possible to fully restore the function of mucociliary transport, as a result ventilation of small bronchi gradually deteriorates, bronchiectasis is observed in the basal segments of the right lung, a polyserial strain S. aureus is formed, which is constantly sown from the child's sputum; it was not possible to fully restore the patency of the nasolacrimal canals and achieve a stable remission of chronic sinusitis. The research was carried out in accordance with the principles of the Helsinki Declaration. The informed consent of the patient was obtained for conducting the studies. No conflict of interest was declared by the authors. Key words: Lyell's syndrome, children, Long-term consequences, chronic obstructive bronchitis, obesity, treatment.

Long-term consequences of Lyell's syndrome suffered in childhood

The article provides a review of literature on the long-term consequences of Lyell's syndrome (one of the forms of toxic epidermal necrolysis). Among them: chronic eczema with xerosis and itching, skin depigmentation, hypertrophic and keloid scars, nail lesions, eyes, including dry eye syndrome and chronic conjunctivitis with/without obliteration of the nasolacrimal canal, kidney disease, lung disease, Sjogren's syndrome, Hashimoto's thyroiditis and others. Clinical case. The authors present their clinical case of an 11-year-old girl who suffered from Lyell's syndrome at the age of 8 and has been observed for the past three years, share their clinical and laboratory-instrumental observations and treatment strategies. In particular, they report about the revision of the clinical diagnosis with which the girl was admitted to the OHMADYT hospital, from BA to chronic secondary obstructive bronchitis, the detection of chronic conjunctivitis with obliteration of the nasolacrimal canals, chronic sinusitis, secondary immune deficiency, keloid skin scars. Conclusions. Despite constant rehabilitation therapy and a significant improvement in the general condition of the girl, it was not possible to fully restore the function of mucociliary transport, as a result ventilation of small bronchi gradually deteriorates, bronchiectasis is observed in the basal segments of the right lung, a polyserial strain S. aureus is formed, which is constantly sown from the child's sputum; it was not possible to fully restore the patency of the nasolacrimal canals and achieve a stable remission of chronic sinusitis. The research was carried out in accordance with the principles of the Helsinki Declaration. The informed consent of the patient was obtained for conducting the studies. No conflict of interest was declared by the authors. Key words: Lyell's syndrome, children, Long-term consequences, chronic obstructive bronchitis, obesity, treatment.

Toxic epidermal necrolysis (Lyell's syndrome) in a patient with HIV infection.

Lyell's syndrome or toxic epidermal necrolysis (TEN) is more common with human immunodeficiency virus (HIV) infection than in the general population. The article describes a clinical case of Lyell's syndrome in an HIV-infected patient who was first prescribed ART in combination with valganciclovir. The diagnosis was made on the basis of characteristic clinical manifestations and the exclusion of another similar pathology. On the background of the therapy, the rash regressed, the areas of damaged skin became epithelialized, the body temperature returned to normal. A differential diagnosis was made with measles, Stevens-Johnson syndrome, staphylococcal infection. Against the background of the therapy, the patient's condition was positive.

Zastosowanie opatrunków srebrowych w leczeniu oparzeń i odmrożeń, zespołu Lyella oraz ran przewlekłych

The skin is the largest organ of the human body and is composed of several layers each having different properties and physiological functions. The loss of skin integrity due to injury or disease can lead to acute physiological and immune disorders which may even result in death. The following properties are taken into consideration when choosing an appropriate dressing for the burn wound: the ability to support epithelialization and granulation, reduction of pain, patient discomfort, and costs. The aim of this study is to evaluate the usefulness of silver dressings in treatment of burns, frostbites, Lyell’s syndrome and chronic wounds. The use of silver dressings in thermal injuries, Lyell’s syndrome and chronic wounds had a significant effect on treatment progress. Silver dressings can be a temporary substitute for the epidermis in burns, frostbites, Lyell’s syndrome and chronic wounds.

Vajra Bhasma, Ayurvedic medicine: a rare and unusual cause of Lyell’s syndrome and its successful management

Toxic epidermolysis necrosis (TEN) or Lyell syndrome is a potentially life-threatening immunological adverse skin disease, which mostly occurs secondary to the intake of an offending drug. It commonly manifests as a widespread exfoliating bullous lesion in skin and mucous membrane mimicking superficial burns and may result in hypovolemic and/or septic shock. Authors report an unusual case of Lyell’s syndrome in a 42-year-old woman, secondary to the intake of Ayurveda medicine ‘Vajra Bhasma’ (Diamond Ash) prescribed by an Ayurveda physician for treatment of her trigeminal neuralgia. After 8th day of continuous medication intake, she had prodromal illness and rapidly developing exfoliative skin lesion extended over 80% of total body surface area, breathing difficulty, dizziness and anuria. The case was successfully managed by timely diagnosis, adequate hydration and administration of immunoglobulins. After 17 days, the skin epithelium regenerated, and she improved clinically with some depigmented lesions at discharge, which were normalised without any sequel during her further follow-up visits in hospital. Identification and withdrawal of the suspected drug, adequate resuscitation and early immunoglobulin administration are critical in management of TEN.

Lyell’s syndrome as a factor which escalates the risk of laryngotracheal stenosis and atresia after intubation

Toxic epidermal necrolysis (TEN) also known as Lyell’s Syndrome is a rare, acute hypersensitivity reaction characterized by cutaneous and mucosal necrosis. Lyell’s syndrome is self limited, but potentially recurrent disease. The mortality for toxic epidermal necrolysis is approaching to 30%.[1] Subglottic stenosis is the most common anomaly of larynx and trachea requiring tracheostomy in pediatric population. Subglottic part of larynx is the narrowest section of laryngotracheal area of airways in childhood.[5] It is estimated that most of subglottic stenosis cases is acquired and developed as a result of injury caused by long-term use of endotracheal tube. A 2-year-old patient admitted to our Clinic of Children’s Otolaryngology after long treatment for TEN because of problems with breathing without tracheostomy tube. Because of the results of bronchofiberoscopy patient has been sent to our Clinic of Children’s Otolaryngology. We performed laryngotracheoscopy which showed transglottic, subglottic and proximal part of tracheal stenosis and complete atresia of trachea above tracheostomy tube. Result of MRI examination was described as complete atresia of trachea which was 4 mm long and located above tracheostomy tube. Our patient was classified for open surgery intervention- partial cricotracheal resection with reconstruction (CTR). Latest improvements about construction of endotracheal tube and rules of procedures reduced frequency of subglottic stenosis to less than 1%.[11] There are no statistics or medical reports about tracheal atresia caused by intubation or Lyell’s syndrome. This situation forced us to recognise Lyell’s syndrome and endotracheal intubation overlapping on each other as a cause of medical state of our patient.

Lyell’s syndrome: a rare case report

Lyell's syndrome or toxic epidermal necrolysis (TEN) is an epidermal detachment of more than 30% of total body surface area, most commonly due an idiosyncratic immune-allergic reaction to a drug. It causes an acute necrosis of the epidermis, with a clinical picture resembling extensive burns. There is also associated mucosal damage and can occasionally develop into a multiorgan having a poor prognosis. Here we report a case of toxic epidermal necrolysis, due to allopurinol intake and its management as in a large burns and stressing the importance of early treatment in an intensive care unit, with high vigil on fluid and electrolyte balance, prevention and treatment of infection with appropriate antibiotics and regular dressings.