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Lyell's syndrome on a burns unit
368
Lyell’s syndrome on a burns unit N. Zoltie, P. Verlende, T. J. O’Neill and A. W. McKenzie East Anglia Regional Bum Unit, West Norwich Hospital, Norwich, UK
Two casesof Lyelrs syndrome (foxicepidennal necrolysis~managed on a bum unif are presented. The various methods of mana~ewrenf are discwsed, and the argumenfs for treating this life-fhreafening condition on an intensive care bum unit are analysed. Burns (1994) 20, (4), 368-370
Introduction Lyell’s syndrome, or toxic epidennal necrolysis, has been described as a fatal, universal, bullous, ulcerative and exfoliative dermatitis’. It can be considered as the most severe of the exfoliative dermatites, the other similar diseases being Stevens-Johnson syndrome and the scalded skin syndrome. In its most severe form it approximates to a 100 per cent superficial bum, and needs specialized management accordingly. We present two patients managed on a
bum unit, describe our treatment, and discuss the rationale for managing these patients on a bum unit in combination with a dermatologist.
Case reports Case 1
A %year-old US Air Force Legal Officer had acute inflammatory bowel disease treated with Azulfidine (a sulphonamide). He suffered a florid cutaneous reaction necessitating admission to the local USAF hospital, where Lyell’s syndrome was diagnosed, and he was transferred to the regional Burns Unit. On admission, he showed classic dermatological signs of Lyell’s syndrome, with total body superficial skin blistering (Figure I), positive Nikolsky sign, and acutely inflamed conjunctiva and oral mucosa (Fipre 2). He was treated with fluids, potassium permanganate baths, nasogastric feeding and steroids. Two weeks after admission epithelial regeneration was virtually complete (Figwe3) and his mouth and eyes were free of complications (Figure 4).
A 16-year-old schoolboy with known atopic eczema presented with a z-day history of a rash, not typical of eczema. He had no history of relevant drug intake. He was treated in a similar way to the previous patient, with fluids, steroids, antibiotics, creams and emollients topically. He responded well over a period of 3 weeks. Skin biopsy was not conclusive of Lyell’s syndrome, but the features and course of the disease were considered sufficient for clinical diagnosis.
Figure 1. Case I demonstrating part of total body superficial skin blistering. 0 19% Butter-worth-Heinemann Ltd 03054179/94/040368-03
Figure 2. Case 1 demonstrating acutely inflamed conjunctiva and perioral area (intraoral mucosa was also severely affected).
Zoltie et al.: Lyell’s syndrome
Figure 4. CaseI two weekslater showing resolution of conjunctival and oral inflammation.
Figure 3. CaseI two weeks later showing epithelial regeneration.
Discussion Toxic epidermal necrolysis was first clearly described in 1926 by Hamilton2 and again in 1940 by Wile and Benson’. Independently, both Lang and Walker in South Africa3, and Lye11 in the UK4 further delineated the condition in 1956, along with its causes and effects. It is a florid cutaneous exfoliative skin reaction, in adults mostly associated with sulphonamide drugs. In its most severe form it is of rapid onset, and leads to desquamation of the entire cutaneous surface, including mucosal surfaces. As might be expected from a condition mimicking a IOO per cent superficial burn, there is a significant mortality rate: if drug induced then a 20 per cent mortality has been reported, rising to 50 per cent in the idiopthaic groups. It is not therefore a disease to be taken lightly, but needs to be treated quickly, effectively and appropriately. Management of the condition has received some attention in the literature, and the treatment tends to fall into two areas, with certain aspects being agreed by all as necessary and thus non-controversial, and other aspects of treatment being hotly debated and thus more controversial. Replacement of lost fluid is a prime requirement, but the type of fluid, rate and route of administration appear to vary from centre to centre. Isolation is necessary for control of cross-infection, and the use of special beds (low air loss or fluidised) helps in management of the delicate and painful skin. Nutritional support is necessary, though the route and nature depend on the mucosal involvement of the mouth and more extended gastrointestinal tract. rf the conjunctiva are involved (as in our first patient intensive eye care is necessary, and of course skilled skin care is paramount. Repeated microbiology and treatment of any infections is required, and clearly it is prudent to stop any medications suspected of causing the reaction. Finally, this is a severe debilitating disease needing intensive hospital care and possible lengthy hospital stay; intensive social and psychiatric support is appropriate both for the patient and his or her family.
Certain aspects of management however remain controversial. The precise fluid regimen for replacement is not universal. Since the fluid loss continues burn resuscitation formulae do not apply and the nature of the fluid lost is not well defined. Saline, plasma and albumin may all be used, but the amount and type may well relate to measured parameters on the individual patients. Classical monitors of fluid replacement, as used in burn centres, are adequate (urine output, biochemical and haematological measures), though as with burns the use of central venous catheters may be necessary but should be avoided if possible due to the risk of infection and also practical difficulties related to attachment of the catheter to the sloughing skin. Systemic antibiotics, as in burns, have mostly been discredited, with specific therapy aimed at specific infections preferred. However, some clinicians still prefer prophylaxis at an early stage, though avoiding sulphonamide drugs of course. The weight of evidence in the literature tends to be against any form of steroid therapy, but again individual clinicians have experience of patients in which they say benefit has been obtained. The precise method of skin care, including dressings, autografting (replacing the blistered skin immediately) or excision and skin grafting have all been widely debated, with no clear conclusions. Most authors assume that the skin loss is superficial, and thus treatment is aimed at preserving the deeper layers of skin to allow normal healing to take place, and the aim of the skin care is to give maximum opportunity for this to occur. The precise method of skin care is thus more dependent on the technique usually used by the clinician treating the patient, and the skills of the nurses working on the unit. Thus in our patients the customary management of the dermatologist (potassium permanganate baths) was used in Case I, although in Case2 the treatment was shaded towards management of the patient’s eczema as well. On consideration of the ideal conditions needed for management of Lyell’s syndrome, most already exist in a bums unit. The only further requirements are the diagnostic skills of the dermatologist, and the medical management and occasional different form of skin care. However, the medical and nursing skills exist, as do the physical requirements of isolation, intensive care, and back up and support. Other authors have discussed thiP, and we feel their conclusions bear repetition, especially in the light of our experience. We believe that toxic epidermal necrolysis, or Lyell’s syndrome, should be managed on a bums unit, by a bums team in conjunction with a dermatologist. The team approach has
370
Bums(1994) Vol. 2O/No. 4
been reported as resulting in a decrease in mortalityg*lO. However, it must be recognized that this has cost consequences, as these patients may be expensive to treat’l. Despite this, we suggest, as have other$, that in the UK
as well we should cooperate with and encourage dermatologists to manage Lyell’s syndrome on burn units.
Acknowledgements This paper was presented at the British Bum Association meeting, Norwich, April 1989.
References I Wile U and Benson JA. Exfoliative dermatitis due to phenobarbital with fatal outcome; report of two cases. Ann Intern Med 1940; 13: 1243. 2 Hamilton ES, Geiger CW and Roth JH. Luminal poisoning with conjunctival residue. ll MedJ 1926; 49: 344. 3 Lang R and Walker J. An unusual bullous eruption. S Afi Med J 1956; 30: 97. 4 Lyell A. Toxic epidermal necroiysis: an eruption resembling scalding of the skin. Br] Dermafoll956; 68: 355.
5 Lyell A. Toxic epidermal necrolysis (the scalded skin syndrome): a reappraisal. Br J hmufol1979; 100: 69. 6 Demling RH, Ellerbe S and Lowe NJ. Bum Unit management of toxic epidermal necrolysis. Arch Sttrg 1978; 113: 758. 7 Editorial. Management of toxic epidermal necrolysis. hcef 1984; ii: 1250. 8 Ward DJ, Krzeminska EC and Tanner NSB. Treatment of toxic epidermal necrolysis and a review of six cases. Burns 1990; 16: 97. 9 Adzick NS, Kim SH, Bondoc CC et al. Management of toxic epidermal necrolysis in a paediatric burn centre. Am J Dis ChiM 1985; 139: 499. 10 Heimbach DM, Engrav LH, Marvin JA et al. Toxic epidennal necrolysis - a step forward in treatment jAA4A 1987; 257: 2171. 11 Jordan MH, Lewis MS, Jeng JG et al. Treatment of toxic epidennal necrolysis by bum units: another market or another threat? J Burn Cure R&&l 1991; 12: 579. Paper accepted 30 November
1993.
Correspondenceshould be addressedto: Mr N. Zoltie, Accident and Emergency Department, Leeds General Infirmary, Great George Street, Leeds LSI XX, UK.
JAMESLAING MEMORIALESSAY The BritishBurn Assoktion has instituteda memorialessay to be awardedannuallyin memoryof JamesEllsworth Laing, Burn Surgeon,foundermemberofthe BritishBurnAssociationand a formerEditorof this Journal. Thereis a prize ofup to ;ESOO for the winning essay.
The subjectfor the tenth essayis: Quality Assurance in Burn Patient Care The essay shouldbe confinedto not morethan 10000 wordsand correspondinglyless if up to 6 Figuresand/orTables are inch&d. The substanceof the essay should not alreadyhave been published since the winning essay will be publishedin this Journal. The essayswill be asses,& anonymously. All personsinterestedin the problemsassociatedwith burninginjuryareeligible to submitan essay. The deadlinefor submissionof an essay (4 copies)is 3 1 December1994. Completed essays and any queries should be sent to: The Secretary of the British Burn Aswc~~~o& Dr J.N. Kearney, Ph.D., Yorkshire Regional Tissue Bank, Piiderfielda General Hospital, Wakefield WFl IDG, West Yorkshire# UK.
The title and author(s)of the winning essay
will be announced
in
April 1995.
Lyell’s syndrome on a burns unit N. Zoltie, P. Verlende, T. J. O’Neill and A. W. McKenzie East Anglia Regional Bum Unit, West Norwich Hospital, Norwich, UK
Two casesof Lyelrs syndrome (foxicepidennal necrolysis~managed on a bum unif are presented. The various methods of mana~ewrenf are discwsed, and the argumenfs for treating this life-fhreafening condition on an intensive care bum unit are analysed. Burns (1994) 20, (4), 368-370
Introduction Lyell’s syndrome, or toxic epidennal necrolysis, has been described as a fatal, universal, bullous, ulcerative and exfoliative dermatitis’. It can be considered as the most severe of the exfoliative dermatites, the other similar diseases being Stevens-Johnson syndrome and the scalded skin syndrome. In its most severe form it approximates to a 100 per cent superficial bum, and needs specialized management accordingly. We present two patients managed on a
bum unit, describe our treatment, and discuss the rationale for managing these patients on a bum unit in combination with a dermatologist.
Case reports Case 1
A %year-old US Air Force Legal Officer had acute inflammatory bowel disease treated with Azulfidine (a sulphonamide). He suffered a florid cutaneous reaction necessitating admission to the local USAF hospital, where Lyell’s syndrome was diagnosed, and he was transferred to the regional Burns Unit. On admission, he showed classic dermatological signs of Lyell’s syndrome, with total body superficial skin blistering (Figure I), positive Nikolsky sign, and acutely inflamed conjunctiva and oral mucosa (Fipre 2). He was treated with fluids, potassium permanganate baths, nasogastric feeding and steroids. Two weeks after admission epithelial regeneration was virtually complete (Figwe3) and his mouth and eyes were free of complications (Figure 4).
A 16-year-old schoolboy with known atopic eczema presented with a z-day history of a rash, not typical of eczema. He had no history of relevant drug intake. He was treated in a similar way to the previous patient, with fluids, steroids, antibiotics, creams and emollients topically. He responded well over a period of 3 weeks. Skin biopsy was not conclusive of Lyell’s syndrome, but the features and course of the disease were considered sufficient for clinical diagnosis.
Figure 1. Case I demonstrating part of total body superficial skin blistering. 0 19% Butter-worth-Heinemann Ltd 03054179/94/040368-03
Figure 2. Case 1 demonstrating acutely inflamed conjunctiva and perioral area (intraoral mucosa was also severely affected).
Zoltie et al.: Lyell’s syndrome
Figure 4. CaseI two weekslater showing resolution of conjunctival and oral inflammation.
Figure 3. CaseI two weeks later showing epithelial regeneration.
Discussion Toxic epidermal necrolysis was first clearly described in 1926 by Hamilton2 and again in 1940 by Wile and Benson’. Independently, both Lang and Walker in South Africa3, and Lye11 in the UK4 further delineated the condition in 1956, along with its causes and effects. It is a florid cutaneous exfoliative skin reaction, in adults mostly associated with sulphonamide drugs. In its most severe form it is of rapid onset, and leads to desquamation of the entire cutaneous surface, including mucosal surfaces. As might be expected from a condition mimicking a IOO per cent superficial burn, there is a significant mortality rate: if drug induced then a 20 per cent mortality has been reported, rising to 50 per cent in the idiopthaic groups. It is not therefore a disease to be taken lightly, but needs to be treated quickly, effectively and appropriately. Management of the condition has received some attention in the literature, and the treatment tends to fall into two areas, with certain aspects being agreed by all as necessary and thus non-controversial, and other aspects of treatment being hotly debated and thus more controversial. Replacement of lost fluid is a prime requirement, but the type of fluid, rate and route of administration appear to vary from centre to centre. Isolation is necessary for control of cross-infection, and the use of special beds (low air loss or fluidised) helps in management of the delicate and painful skin. Nutritional support is necessary, though the route and nature depend on the mucosal involvement of the mouth and more extended gastrointestinal tract. rf the conjunctiva are involved (as in our first patient intensive eye care is necessary, and of course skilled skin care is paramount. Repeated microbiology and treatment of any infections is required, and clearly it is prudent to stop any medications suspected of causing the reaction. Finally, this is a severe debilitating disease needing intensive hospital care and possible lengthy hospital stay; intensive social and psychiatric support is appropriate both for the patient and his or her family.
Certain aspects of management however remain controversial. The precise fluid regimen for replacement is not universal. Since the fluid loss continues burn resuscitation formulae do not apply and the nature of the fluid lost is not well defined. Saline, plasma and albumin may all be used, but the amount and type may well relate to measured parameters on the individual patients. Classical monitors of fluid replacement, as used in burn centres, are adequate (urine output, biochemical and haematological measures), though as with burns the use of central venous catheters may be necessary but should be avoided if possible due to the risk of infection and also practical difficulties related to attachment of the catheter to the sloughing skin. Systemic antibiotics, as in burns, have mostly been discredited, with specific therapy aimed at specific infections preferred. However, some clinicians still prefer prophylaxis at an early stage, though avoiding sulphonamide drugs of course. The weight of evidence in the literature tends to be against any form of steroid therapy, but again individual clinicians have experience of patients in which they say benefit has been obtained. The precise method of skin care, including dressings, autografting (replacing the blistered skin immediately) or excision and skin grafting have all been widely debated, with no clear conclusions. Most authors assume that the skin loss is superficial, and thus treatment is aimed at preserving the deeper layers of skin to allow normal healing to take place, and the aim of the skin care is to give maximum opportunity for this to occur. The precise method of skin care is thus more dependent on the technique usually used by the clinician treating the patient, and the skills of the nurses working on the unit. Thus in our patients the customary management of the dermatologist (potassium permanganate baths) was used in Case I, although in Case2 the treatment was shaded towards management of the patient’s eczema as well. On consideration of the ideal conditions needed for management of Lyell’s syndrome, most already exist in a bums unit. The only further requirements are the diagnostic skills of the dermatologist, and the medical management and occasional different form of skin care. However, the medical and nursing skills exist, as do the physical requirements of isolation, intensive care, and back up and support. Other authors have discussed thiP, and we feel their conclusions bear repetition, especially in the light of our experience. We believe that toxic epidermal necrolysis, or Lyell’s syndrome, should be managed on a bums unit, by a bums team in conjunction with a dermatologist. The team approach has
370
Bums(1994) Vol. 2O/No. 4
been reported as resulting in a decrease in mortalityg*lO. However, it must be recognized that this has cost consequences, as these patients may be expensive to treat’l. Despite this, we suggest, as have other$, that in the UK
as well we should cooperate with and encourage dermatologists to manage Lyell’s syndrome on burn units.
Acknowledgements This paper was presented at the British Bum Association meeting, Norwich, April 1989.
References I Wile U and Benson JA. Exfoliative dermatitis due to phenobarbital with fatal outcome; report of two cases. Ann Intern Med 1940; 13: 1243. 2 Hamilton ES, Geiger CW and Roth JH. Luminal poisoning with conjunctival residue. ll MedJ 1926; 49: 344. 3 Lang R and Walker J. An unusual bullous eruption. S Afi Med J 1956; 30: 97. 4 Lyell A. Toxic epidermal necroiysis: an eruption resembling scalding of the skin. Br] Dermafoll956; 68: 355.
5 Lyell A. Toxic epidermal necrolysis (the scalded skin syndrome): a reappraisal. Br J hmufol1979; 100: 69. 6 Demling RH, Ellerbe S and Lowe NJ. Bum Unit management of toxic epidermal necrolysis. Arch Sttrg 1978; 113: 758. 7 Editorial. Management of toxic epidermal necrolysis. hcef 1984; ii: 1250. 8 Ward DJ, Krzeminska EC and Tanner NSB. Treatment of toxic epidermal necrolysis and a review of six cases. Burns 1990; 16: 97. 9 Adzick NS, Kim SH, Bondoc CC et al. Management of toxic epidermal necrolysis in a paediatric burn centre. Am J Dis ChiM 1985; 139: 499. 10 Heimbach DM, Engrav LH, Marvin JA et al. Toxic epidennal necrolysis - a step forward in treatment jAA4A 1987; 257: 2171. 11 Jordan MH, Lewis MS, Jeng JG et al. Treatment of toxic epidennal necrolysis by bum units: another market or another threat? J Burn Cure R&&l 1991; 12: 579. Paper accepted 30 November
1993.
Correspondenceshould be addressedto: Mr N. Zoltie, Accident and Emergency Department, Leeds General Infirmary, Great George Street, Leeds LSI XX, UK.
JAMESLAING MEMORIALESSAY The BritishBurn Assoktion has instituteda memorialessay to be awardedannuallyin memoryof JamesEllsworth Laing, Burn Surgeon,foundermemberofthe BritishBurnAssociationand a formerEditorof this Journal. Thereis a prize ofup to ;ESOO for the winning essay.
The subjectfor the tenth essayis: Quality Assurance in Burn Patient Care The essay shouldbe confinedto not morethan 10000 wordsand correspondinglyless if up to 6 Figuresand/orTables are inch&d. The substanceof the essay should not alreadyhave been published since the winning essay will be publishedin this Journal. The essayswill be asses,& anonymously. All personsinterestedin the problemsassociatedwith burninginjuryareeligible to submitan essay. The deadlinefor submissionof an essay (4 copies)is 3 1 December1994. Completed essays and any queries should be sent to: The Secretary of the British Burn Aswc~~~o& Dr J.N. Kearney, Ph.D., Yorkshire Regional Tissue Bank, Piiderfielda General Hospital, Wakefield WFl IDG, West Yorkshire# UK.
The title and author(s)of the winning essay
will be announced
in
April 1995.