INTRODUCTION
Toxic epidermal necrolysis (TEN), also known as Lyell’s syndrome (LS), is an allergic
reaction mediated by CD8 lymphocytes that evolves with epidermal necrosis due to keratinocyte
apoptosis. The etiology is unknown, but the use of drugs triggers it. Due to the high
mortality, knowledge of the condition, early diagnosis, stratification, and an adequate
approach is essential for optimized patient management.
CASE REPORT
Patient MD, woman, 28 years old, mixed race, from the municipality of Laranjal do
Jari in the state of Amapá. On January 26, 2019, the patient presented phlyctenas
in the oral mucosa after using the medication allopurinol 200 mg per day for 17 consecutive
days, 50 mg of diclofenac sodium, 30 mg of caffeine, 125 mg of carisoprodol, 300 mg
of paracetamol every 12 hours. In the following 48 hours, the patient developed phlyctenas
on 72% of the body surface. Involvement was also observed in the mucosa of the oral,
vaginal, anal, and genitourinary tracts.
In the first 48 hours, her body temperature was 38ºC, and she was admitted to the
Hospital Estadual de Laranjal do Jari on January 28, 2019. On February 1, 2019, she
was referred to the Hospital de Emergência Osvaldo Cruz, where an evaluation was requested
from the plastic surgery team (Figure 1). Upon entering the plastic surgery service, she was treated clinically, showing
a satisfactory evolution, being discharged without the need for surgical treatment
on February 10, 2019 (Figure 2). The SCORTEN scale was not calculated as it was not applied at the time of disease
progression.
Figure 1 - Patient no 17º of evolution no day of admission no burn center.
Figure 1 - Patient no 17º of evolution no day of admission no burn center.
Figure 2 - Patient on the day of hospital discharge.
Figure 2 - Patient on the day of hospital discharge.
DISCUSSION
In 1922, Indian surgeon Albert Stevens and American pediatrician Frank Johnson described
two cases of fever, conjunctivitis, and inflammation of the mucous membranes in children,
with one case with total loss of vision. Despite the lack of knowledge of the cause
at the time, it was the first description of what was later called Stevens-Johnson
Syndrome (SJS). Back in 1956, Scottish dermatologist Alan Lyell described a condition
in which disseminated epidermal necrosis occurred, which evolved with the formation
of phlyctenas associated with a toxic febrile reaction. The pathology study was of
apoptosis of keratinocytes, which the dermatologist named TEN1.
Despite the low incidence: 2 cases per million per year, mortality from the disease
is high: 30%. It is a hypersensitivity reaction that affects the skin and mucous membranes
and does not yet have a clear etiology. The leading cause of TEN is medication, and
the primary triggers are allopurinol, aromatic anticonvulsants, sulfonamides, and
non-steroidal anti-inflammatory drugs. It is also seen more frequently in patients
with HIV, systemic lupus erythematosus, and patients undergoing bone marrow transplantation.
On average, the exposure time to the trigger factor is two weeks, but there are reports
of up to 48 hours2.
Didactically TEN can be divided according to the affected body surface. In this way,
it is called SJS in cases where the participation is up to 10%; overlap syndrome when
involvement varies between 10 and 30%; and TEN, also known as Lyell Syndrome (LS),
when the participation exceeds 30%3. Due to the high mortality, the SCORTEN scale - Disease severity score was developed
for the toxic epidermal necrolysis scale. On the scale, seven variables weigh one
point each and are: age over 40 years; heart rate higher than 120 beats per minute;
associated malignancy; epidermal detachment of more than 10% of the body surface on
the first day; urea greater than 28 mg/dl; glucose higher than 252 mg/dl and bicarbonate
less than 20 mEq /l. According to the score, mortality increases dramatically (Table 1). The scale should be applied on the first and third days of hospitalization to increase
the predictive value4.
Table 1 - SCORTEN scale - Severity of Illness Score for Toxic Epidermal Necrolysis.
Risk factors |
Age> 40 years |
Neoplasm |
Heart rate> 120bpm |
Epidermis detachment> 10% |
Urea> 28md / dl |
Glucose> 252mg / dl |
Serum bicarbonate <20mg / dl |
Mortality |
Scorten 0 or 1 |
3,2% |
Scorten 2 |
12,1% |
Scorten 3 |
35,3% |
Scorten 4 |
58,3% |
Scorten > 4 |
90% |
Table 1 - SCORTEN scale - Severity of Illness Score for Toxic Epidermal Necrolysis.
The initial manifestation can be confused with a flu-like syndrome for three days
before the mucocutaneous manifestations. On physical examination, the Nikolsky and
Asboe-Hansen sign can be seen: shear of the skin with light friction and lateral shedding
of the skin after light pressure on the blister, respectively. The involvement of
the urethral, genital, oral, and ocular mucosa is frequent and can precede skin
lesions3.
Treatment consists of removing the causative agent and offering the necessary support
to the patient, preferably in a burn treatment unit. There is no consensus on the
best way to manage wounds, as some ointments can trigger a similar condition so that
injuries can be treated with soap and water. Surgical debridement is also not a consensus.
Systemic antibiotics should be reserved only for cases of infection, as they can also
be causative agents of TEN. The use of intravenous corticosteroids has not shown benefits
and may even delay the healing process and favor secondary infection. Plasmapheresis,
although indicated by some authors, did not show a significant impact on mortality
and length of stay. The use of cyclosporine has been indicated successfully, although
the mechanism of action is not yet well defined. The use of immunoglobulins has shown
good results and depends on the dose and early administration5.
REFERENCES
1. Lyell A. Toxic epidermal necrolysis: an eruption resembling scalding of the skin.
Br J Dermatol. 1956 Nov;68(11):355-61. DOI: http://dx.doi.org/10.1111/j.1365-2133.1956.tb12766.x
2. Santos Neto FC, Piccinini PS, Andary JC, Sartori LDP, Cancian LT, Uebel CO, et al.
Abordagem cutânea na necrólise epidérmica tóxica. Rev Bras Cir Plást. 2017;32(1):128-34.
3. http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365- 05962004000400009&lng=en
http://dx.doi.org/10.1590/S0365-05962004000400009
4. Trent JT, Kirsner RS, Romanelli P, Kerdel FA. Use of SCORTEN to accurately predict
mortality in patients with toxic epidermal necrolysis in the United States. Arch Dermatol.
2004 Jul;140(7):890-2. DOI: http://dx.doi.org/10.1001/archderm.140.7.890
5. https://www.scielo.br/scielo.php?pid=S0104-42302016000500468&script=sci_abstract http://dx.doi.org/10.1590/1806-9282.62.05.468
1. Universidade Federal do Amapá, Macapá, AP, Brazil.
2. Hospital de Emergência Osvaldo Cruz, Macapá, AP, Brazil.
Corresponding author: José Augusto Pupio Reis Júnior Rodovia JK, KM 6, n 4440, Bairro Universidade, Macapá, AP, Brazil. Zip Code: 68903-419
E-mail: alieksei@gmail.com
Article received: February 22, 2019.
Article accepted: October 20, 2019.
Conflicts of interest: none.