Annals of*the MBC - vol. 5 - n' 3 -
BURN UNIT TREATMENT
OF THREE STEVENS-JOHNSON SYNDROME CASES WITH CRYOPRESERVED ALLOGRAFT
Pousa F., Valero J., Vazquez-Barro A., Trincado S.
Burns Unit, Juan Canalejo Hospital, La Coruha, Spain
Three cases of Stevens-Johnson syndrome are described. This syndrome is characterized by
extensive loss of tffe epidermis and other complications. The cutaneous lesions are
similar to second-degree burns in their development, and they benefit from temporary
coverage with cryopreserved allografts, as may be performed in a Burns Centre.
Burns Units are referral centres for the treatment of patients suffering from considerable
cutaneous loss(1,2), sometimes aetiologically distinct from burns, such as those caused by
disseminated intravascular coagulation, traumatic avulsions and exfoliative dermAtitis
complaints, among which the Stevens-Johnson syndrome (S.J.S.) is significant.
S.J.S. has an acute, self-limiting systemic clinical setting characterized by extensive
and painful loss of the epidermis with skin, mucous membrane and corneal involvement,
accompanied by general symptoms such as tachycardia and fever (3). Nutritional
requirements are augmented, as are fluid losses via the lesioned skin.
The cutaneous lesions, equivalent to seconddegree burns in their development and treatment
(1,4), benefit from the provision of a suitable temporary coverage with cryopreserved
A 17-year-old female, weighing 56 kg, presented an exfoliative dermatitis affecting
95% of the total body surface. The clinical setting had started ten days previously with a
reddish macular exanthem in areas exposed to light (face, neck and hands). On these
exanthematic lesions vesicles with a serous content appeared which developed into
blisters, leading to an extensive loss of epidermis. There was no past history of drug
taking, viral infection or recent vaccinations. On admission, besides the cutaneous
lesions, there were also severe lesions of the oral, conjunctival and vaginal mucous
membranes and a temperature of 40 'C. High doses of corticosteroids and systemic
prophylactic antibiotics were administered. Analysis showed granulocytopenia-derived
leukopenia and slight normocytic/normochromic anaemia. Haemocultures were methicil lin
-resistant Staphylococcus aureus positive; cutaneous cultures obtained from wounds in the
trunk and extremities were Staphylococcus epidermidis, Pseudomonas aeruginosa, Klebsiella
pneumoniae and enterococcus positive. A pre-admittance biopsy of skin from the affected
areas showed the existence of subepidermic vesicles with a necrotic epidermis ceiling and
a denuded, festinated papillary dermis floor, with perivascular distribution of
mononuelear inflammatory infiltration, all characteristic of S.J.S.
Treatment was based on gradual elimination of the corticosteroids, initiation of
prophylaxis of the stress ulcers with oral sucralphate, and nutritional support with oral
supplements. Empiric treatment of the sepsis with aztreonam and vancomycin was effective
and unmodified thereafter.
Among other steps taken were the removal of a central venous catheter, substituted by a
peripheral catheter, and the removal of the bandages. Zinc sulphate was applied topically.
The cutaneous lesions of the rear trunk and upper extremities worsened 72 hours after
admission, necessitating surgery under general anaesthesia for the debridement and
coverage of extensive areas with cryopreserved allografts, maintaining exposure after the
operation. The general condition improved, as did granulocyte recounts as leukopenia
disappeared. Eight days after surgery the trunk and extremities were epithelialized. The
patient was released 11 days after surgery.
A 7-year-old female, weighing 28 kg, was admitted five days after the onset of a
generalized maculovesicular exanthem which began on the thorax and abdomen and extended to
the face and extremities, affecting 65% TBSA. The lesions were accompanied by fever, but
no other systemic symptoms, and slight irritation of oral, vaginal and rectal mucosae and
ocular conjunctivae. Treatment with methylprednisolone, total parenteral nutrition via the
right subclavian artery and antibiotic prophylaxis with cefotaxime and cloxacillin had
been started on admittance to hospital before transfer to this Unit. Analysis showed
severe leukopenia but apart from the temperature nothing indicated sepsis. Biopsy of skin
from the aftected areas confirmed the clinical diagnosis. The corticosterolds were
gradually eliminated and the prophylactic antibiotherapy suspended. Total parenteral
nutrition was suspended and the central catheter removed, being replaced by oral dietetic
supplements. Topical therapy consisted of debridement of the aftected areas and coverage
using cryopreserved allografts. The cutaneous lesions and leukopenia reacted favourably
and the patient was released 15 days after admission.
A 75-year-old man developed an exfoliative dermatitis 10 days after taking allopurinol for
hyperuricaemia; 64% TI1SA was involved, mainly on the limbs and thorax. The oral mucosa
and the cornea were also involved. He was transferred to the Burns Unit five days after
developing the rash, with a treatment based on prophylactic antibiotics, steroids an
After admission the steroids were gradually withdrawn, the antibiotics were removed,
prophylaxis of the stress ulcers with sucralphate was performed, the central venous
catheter was replaced by a peripheral one and nutritional support was given with oral
A skin biopsy specimen taken to determine the level of epidermal separation showed
necrosis of the upper 4 to 5 epidermal cell layers, with cleavage occurring mainly at the
suprabasal epidermal layer.
The wounds were treated initially with zinc sulphate, and 24 hours after admission with
debridement and coverage with cryopreserved allograft under general exposure after the
Fifteen days after admission, when the areas of skin loss were completely
re-epithelialized, the patient developed pneumonia and urinary infection. Empiric
antibiotic treatment was initiated (imipenem and tobramycin). The infections were caused
by Pseudomonas aeruginosa. The patient died of pulmonary failure 30 days after
admission. anaesthesia, maintaining
The Stevens-Johnson syndrome is an acute, self-limiting systemic process characterised by
painful, extensive loss of the epidermis affecting the skin, mucous membranes and corneas
(5), accompanied by systemic symptoms such as fever and tachycardia (2,3,6,7). In 1957
Lyell related this clinical setting to exposure to several drugs such as the
sulphonamides, phenobarbital, diphenylhydantoin, penicillin G sodium and penicillin G
potassium, exposure to a staphylococcal toxin and also exanthematic viral diseases and
their vaccines, no aetiological factors being found in 27% of cases (5,6,7).
In 1967 Koblenzer defined as a different entity the previously denominated Staphylococcic
Syndrome (Scalded Skin Syndrome) (6), based on the fact that the location of the bullae
was not in the dermoepidermal junction, as in cases of S.J.S., but in the upper layer of
the epidermis(5,8). Mortality is lower (3% approximately) in the Staphylococcic Syndrome
thanks to the efficacy of the antistaphylococcics (1,3,4,5,6,7,9).
S.J.S. is a severe form of erythema multiforme which most frequently affects children and
young adults. Patients may present a prodromal period, characterized by fever, malaise and
pharyngitis before eryt ema, although in severe cases these prodromes may be absent, as in
the cases we report. Erythema usually appears on the lips and face as a diffuse redness or
as dark papules with a red margin. 24 to 48 hours later blisters appear, and we observe a
positive Nicholsky's sign and a temperature of 38-40 'C, with systemic toxic reactions,
conjunctivitis and involvement of oral, vaginal, digestive and upper airway mucous
membranes. Renal involvement develops in more than 50% of patients, usually due to
hypovolaemia which causes acute tubular necrosis, but occasionally to progressive
membranous glomerulonephritis or membranoproliferativeness, even several years after the
acute episode (4,6,8, 10). It was difficult to ascertain if there was severe renal
dysfunction in our patients, owing to their late admission. Pneumonia is common but can be
treated successfully by conventional means (3,6).
If the patient lives, epithelialization from skin appendages is achieved in 10 to 28 days,
but in some cases there are scars, contractures, oesophageal, urethral or vaginal
strictures, permanent loss of nails or intestinal pneumatosis (7,11).
The patients we report were admitted to the Burn Unit as the complications deriving from
the cutaneous lesions are similar to those of extensive second-degree burns, i.e. massive
hydroelectrolytic loss, breakdown of thermic regulation, increase in catabolism, and the
abolition of cutaneous antimicrobial protection. Although there is great fluid loss,
resuscitation is less important than in the case of bums, perhaps due to the fact that
there is no intense oedema secondary to the release of acute phase reactants, as occurs in
The late admission of the cases reported is normal, given the difficulties of making
medical personnel aware of the need to refer cases early in order to avoid lesion
contamination, and to facilitate adequate coverage as soon as possible (1). Patients are
admitted 5-6 days after the first symptoms, with extensive exfoliative lesions, frequently
septic, and undergoing corticosteroid treatment. On admission central venous catheters are
removed and replaced by peripheral ones, in order to reduce the risk of sepsis by catheter
(3,4). Blood, catheter and wound cultures and lesion biopsies provide an accurate
Although for years high doses of corticosteroids have been used to check the progress of
cutaneous lesions, to improve systemic toxicity signs and symptoms and to reduce
temperature, it is now generally held that their use is erroneous as gastrointestinal
ulcerations, Candida albicans and polymicrobial gram-negative septicaemia occur
less frequently when they are not used. For this reason they are gradually suspended on
admission to our unit.
Topical therapy consists of the application of zinc sulphate up to the time of coverage
with cryopreserved allografts, although there are no studies which rigorously uphold its
use. We do not believe in the use of gentamicin and other topical antibiotics because of
the resistance and absorption risk which they present (4,6), or in the use of commercial
preparations with polyethylene glycol, such as nitrofurazone, proved to be toxic when used
for extensive burns. Perhaps the most used chemotherapeutic treatment in Bums Units is
silver sulphadiazine, as it is easy to apply and has few sideeffects. It is not advisable
in these cases, however, due to its relationship with the onset of the illness (8,12).
Some authors recommend bandages soaked in silver nitrate 0.5%. These do not produce
allergic reactions and do not inhibit epithelialization, but they can cause severe
hyponatraemia if used extensively. They are also inconvenient, as they stain bedding and
must be changed every 12 hours (2).
Fundamental to this discussion is the restriction in the use of the prophylactic
antimicrobials even in the presence of leukopenia (3). Granulocytopenia causes leukopenia
and its persistence leads to 'an elevated risk of infection, which is not well understood
(2,4,5,6,7). It is thought that the greater incidence of septic complications in S.J.S.
than in burns is perhaps due to the immunological defect, the involvement of mucous
membranes, leukopenia, and epidermic necrosis.
Treatment includes measures normally taken in Burns Unit, such as nutritional support and
dietetic supplements per os in order to avoid nasogastric tubes (1,3,7).
The treatment most specifically related to a Burns Unit with access to a skin bank was,
for these patients, the use of cryopreserved allografts. At the present time, this is the
closest to the ideal coverage available, as it reduces pain, hydroelectrolytic cutaneous
losses and bacterial multiplication, improving thermoregulation and promoting
reepithelialization (1,5). It is applied under general anaesthesia, avoiding endotracheal
intubation so as not to increase damage to the upper airways which are often involved in
the syndrome, after an operative debridement to slough-detached epithelium. If this
biological dressing is unavailable, amnion or porcine cutaneous xenografts or skin
substitutes may be used (1,3,7,8,13).
Two of these cases healed well, even though affected in more than 50% T13SA, which,
together with the afore-mentioned granulocytopenia, would indicate a poor prognosis
(5,10,12); with the exception of evident scars on the trunk of one patient, severe
after-eflects were not seen at subsequen~ check-ups.
In conclusion, Burns Units provide specially trained personnel for topical care, intensive
monitorization and the most aseptic environment possible, thereby of necessity improving
results with S.J.S. patients.
RESUME. Les autevrs décrivent
trois cas du syndrome de Stevens-Johnson, qui est charactérisé par la perte étendue de
1'&piderme et d'autres complications. Les 1ésions cutanées sont semblables aux
brOlures du second degré dans leur développement, et elles bénéficient d'une
couverture momentanée avec des allogreffes cryopréservées, qui peut &tre effectuée
chez un centre de brùlés.
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