Toxic epidermal necrolysis (TEN) is a rare but very impressive disease, especially in children. “It is acute and fast. That is very exciting for these people,” says nurse specialist José Duipmans. “Also for the family”, adds Prof. Barbara Horváth. “Someone gets seriously ill before their eyes. There is a lot of haste and panic.” Therefore, rapid recognition and referral of these patients is essential. This also applies to the correct nursing technique, specifically wound care.
The UMCG is a center of expertise for blistering diseases. “We have a lot of experience in connecting people with extensive skin detachment”, says Duipmans. “That is partly why we are suitable to properly treat patients with TEN and to provide supportive care during admission.” Horváth agrees that supportive care is extremely important.
Walk with IC recording
TEN is a rare condition in which large parts (> 30%) of the skin and mucous membranes peel off. The incidence is about 1 or 2 cases per million people per year. The main triggering factor is drugs. Prior to the epidermolysis, the patient has flu-like symptoms for 1 or 2 days, such as general malaise, fever, muscle aches and joint pain. Then the epidermis dies and the epidermal layer of the skin and mucous membranes loosens and erosions appear. The patient feels very ill and experiences a lot of pain.1 Eating, drinking, lying down, swallowing are no longer possible.
The diagnosis of TEN is confirmed with an emergency skin biopsy using frozen sections. These patients should be admitted immediately, preferably in an intensive care unit (ICU) or in a burn center. All aspects of burn treatment must be taken into account, such as wound care, tube feeding, fluid and electrolyte balance and infection control (see box).2
Experiences from Rotterdam1
Nursing care is an important part of the treatment of TEN patients. Unfortunately, there is little information available in the current literature about the care of these patients. In a retrospective study, published in 2019, data were collected from the nursing records of all patients with TEN (>30% detachment), Stevens-Johnson syndrome (SJS, < 10% loslating) of SJS-TEN-overlap (10-30% loslating) die tussen 1987 en 2016 in het Brandwondencentrum Rotterdam waren opgenomen. Van 59 van de 69 gevonden patiënten waren de patiëntendossiers beschikbaar. De meest gemelde verpleegkundige problemen, die aanwezig waren bij > 20% of the patients were wounds, threatened or impaired vital functions, dehydration, fluid imbalance, pain, secretion problems and fever.
Furthermore, TEN-specific nursing problems were documented, including lesions of the oral mucosa and eye problems. The highest number of concomitant nursing problems occurred between days 3 and 20 after disease onset and varied by nursing problem.
With this knowledge we can start nursing interventions early in treatment, address problems at the first sign and inform patients and their families about these problems early in the disease process. A next step to improve nursing care for TEN patients is to implement the knowledge about the optimal interventions for nursing problems.
Acute course requires rapid recognition
Due to the serious and acute course of TEN, rapid recognition is necessary. “We aim to have a diagnosis within 1 hour, with frozen sections,” Horváth says. “A pathologist is needed for that analysis, who may examine the sections at night. That is well organized in our region; every hospital can perform emergency diagnostics here. Sometimes the pathologist mistakenly diagnoses TEN (false positive result) in fear of overlooking this disease. We link that back to each other, so we learn from each other. You always try to think along with colleagues, for example to look for a different diagnosis if there is no TEN.”
In order to act quickly and effectively, it is extremely important to be well prepared. “Everyone is quick to respond to the emergency department (ED), says Horváth about the working method at the UMCG. “We try to arrange the transport of these patients properly. Preferably there is consultation with one of our nurse specialists in blistering disease prior to transport. The pathologist, internist and the Martini Hospital are aware.”
“Once the diagnosis of TEN has been made and the patient is with us, we immediately start with the right wound care,” adds Duipmans. “If more than 20% of the skin is affected, the patient is admitted to the Burn Center of the Martini Hospital. Otherwise, the patient stays with us and we do an extensive dressing change 3 to 5 times a week. An extensive team of specialists, such as ENT specialist, ophthalmologist, internist, dental hygienist and pain specialist, are involved in the treatment.”
Grease gauze and non-adhesive dressings
What sometimes happens when patients end up in the UMCG after a referral is that they are connected to unsuitable materials or fat gauze that is not properly covered. “Then the bandage is stuck in the wound, after which the removal becomes very painful,” says Duipmans. “At that time, patients built up a lot of fear about the dressing change. You have to regain trust. You can reassure the patient by saying that after good bandaging, there is less discomfort. In addition, the patient can move and breathe better again. After covering the open skin, you will pay attention to the mucous membranes of the eyes, ears, nose and genitals. Black scabs on lips and eyes are also gently removed.”
Duipmans and colleagues cover the wounds with non-adhesive bandages. “Because we have experience with other blister diseases, we like to use silicone foam dressings. There are also other good options. It is important that the product does not get stuck in the wound. If you were to use fat gauze, you could apply a double layer, or you would add extra fat.”
If the skin is not open, but is loose, Duipmans and colleagues also cover those areas. “Experience has shown that preventive covering of loose, non-erosive skin causes less pain and discomfort. After all, if your skin is loose, you feel that your body’s defenses are not working properly. It’s painful and you literally feel vulnerable. Covering up gives the patient a new skin, as it were.”
wound care
Taking care of the skin and mucous membranes of TEN patients presents many challenges. Large areas are often affected, including areas that are difficult to connect, such as armpits and groin. “Open mucous membranes are also difficult to care for,” Duipmans knows from experience. “The genitals must also be kept open, because there is a risk of adhesions. You have to rub the foreskin of the penis well. You have to take care of the mucous membranes every 2 hours. Crusts, dry skin and flakes are also taken care of. Furthermore, no adhesive materials should come on the skin. They would take the skin with them when picked up. This means that for the fixation of materials you have to use tricks with net and tube bandages, and self-adhesive bandage.” We always carry out a dressing change with 2 people, adds Duipmans. “It is important to provide pain relief before the dressing change and to ensure that all materials are ready. However, after an extensive dressing change, everyone is tired, the Groningen nurse specialist knows from practice. Over a cup of coffee, we discuss with the patient what went well and what could be improved.”
Pain relief and other advice
The wound care of TEN patients is not possible without pain unless the patient is given general anaesthesia. In the UMCG, these patients are rarely connected in this way, because only patients with limited skin detachment remain in the UMCG. The pain team is called in for pain relief, both for pain throughout the day and during dressing changes. Horváth agrees with the major impact of pain: “It is very important to transport these patients in the right way. The very first thing said in the ER is: pain relief.”
“In addition, you have to ensure a good room temperature, protective insulation, ventilation and possibly later, if the patient’s condition permits, bathing in isotonic bath water, so that it hurts less,” says Duipmans. “You have to do everything you can to increase comfort.”
Psychological trauma and other problems
In addition to (wound) care in the acute phase, healthcare providers must be aware of the long-term consequences of this disease. In the discharge letter to the general practitioner and to other doctors involved, all possible symptoms that may occur should therefore be mentioned. “Also pay particular attention to the psychosocial aspects of the patient”, advises Horváth. “Many of these people have a psychological trauma. After discharge, they are followed for a long time by a psychologist. They are very afraid to take pills.”
Patients from the region regularly come to the UMCG for check-up appointments with, among others, the ophthalmologist and the oral surgeon. They can have many consequences of a previous TEN, such as conjunctivitis due to dry eyes or caries due to dry mouth. “Furthermore, they can no longer sweat properly and develop pigmentation spots,” adds Duipmans. “And they sometimes temporarily lose their nails.”
Long Term Consequences
It is difficult to determine the long-term consequences of TEN, especially because many of these patients become invisible. There are several reasons for this, Horváth says. “These are often elderly patients and the disease is usually triggered by medication. Often patients have died after a longer period of time, but because of something other than TEN. In addition, because we are a rural center, many patients come from far away. They are referred back to their own dermatologist.”
Horváth does think it is important to monitor the complications of this disease. The long-term effects of this disease are being mapped out in an ongoing study of the burn centers.
References
- Trommel N, Hofland HW, van Komen RS, et al. Nursing problems in patients with toxic epidermal necrolysis and Stevens-Johnson-syndrome in a Dutch burn centre: A 30-year retrospective study. Burns. 2019;45:1625-1633.
- Njoo MD. Toxic Epidermal Necrolysis (TEN)/Lyell’s Syndrome. www.skinarts.com.