Case Report

Steven-Johnson syndrome a case

report
Ramachandran Sudarshan,* Rajeshwari G Annigeri, Sree
Vijayabala
*Senior Lecturer, Department of Oral Medicine & Radiology, Sibar Institute of Dental
Sciences, Guntur, India. Professor and Head, Department of Oral Medicine & Radiology,
College of Dental Sciences & Hospital, Davangere, India. Senior Lecturer, Department of
Oral Medicine & Radiology, Thai Moogambikai Dental College and Hospital, Chennai, India

Abstract
Physicians writing prescriptions for patients must be careful regarding
the adverse effects of the drugs especially the one is the StevensJohnson syndrome (SJS), a potentially fatal condition that manifests
not only as mucocutaneous disorder but also affects vital organs. We
report a case of Steven Johnson syndrome with mucocutaneous
manifestation due to amend of the drug. Care for patients with
Stevens-Johnson syndrome consists of treating the presenting
symptoms, electrolyte balance, sketch and confiscation of the drug
which manifest this condition in the patient.
Key words:
Stevens-Johnson syndrome; Erythema Multiforme;
Mucocutaneous disorder.

Introduction
The term Erythema Multiforme (EM) includes a wide range of
clinical expressions, from exclusive mucous or skin erosions to
mucocutaneous lesions (EM minor) and, in its more severe forms,
there is a serious involvement of multiple mucosal membranes and
skin (EM major, Stevens-Johnson Syndrome) or a large area of the total
body surface including mucous surfaces (Toxic Epidermal Necrolysis)
with constitutional symptoms and, at times, visceral involvement (1).
Stevens-Johnson syndrome, otherwise known as erythema multiforme
majus, is thought to represent a continuum of disease, the most benign
type of which is erythema multiforme, whereas toxic epidermal
necrolysis is the most severe (2).

*Author for correspondence: Dr. Sudarshan R, Senior lecturer, Department of Oral

Medicine and Radiology, Sibar Institute of Dental Sciences, Guntur, India.
Tel: +91-7416433735
E-mail: ssudharshanram@yahoo.co.in

Steven Johnson syndrome

Steven - Johnson syndrome is an uncommon
mucocutaneous disorder. The etiology of
Steven - Johnson syndrome is frequently
unknown, but many cases are associated
with recent drug exposure or infection with
pathogens such as herpes simplex virus or
mycoplasma (3).

had a central dark area and peripheral round

erythematous area (figure 1).

Case report
An 18 year old male patient reported
to our department with the chief complaint
of burning sensation and soreness of mouth
since three days. Patient was apparently
normal a week ago after which he developed
burning sensation, stickiness of eyes and
scanty mucopurulent discharge three days
ago and two days later he developed soreness
of mouth, difficulty in swallowing and
burning sensation on consumption of food.
His medical history revealed epilepsy from
past 10 years for which he is on phenytoin
since 10 years. He had history of intermittent
fever for which he had been to a local doctor
7 days ago for which he was prescribed some
medication followed by which fever
subsided, in addition medication for epilepsy
was
changed
from
phenytoin
to
phenobarbitone. Patient gave history of
similar complaint two years ago.

Figure 1: Target lesions

On cutaneous examination, there
were generalized asymmetric target lesions
on forearm, legs and chest that were round
in shape with well-defined borders which

Figure 2: Purulent conjunctivitis

Figure 3: Ruptured vesicles and bullae

On mucosal examination, there was
purulent discharge in the eyes (figure 2).
Erosions were present in the penile mucosa.
Erosions were also seen in the labial mucosa
and hemorrhagic crusts were present over
the swollen lips involving the vermilion
border thereby hindering phonation (figure
3).

Figure 4: Healed lesion

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48

Steven Johnson syndrome

Generalized marginal and attached
gingival enlargement was present. Intact
vesicles and bullae were also present on the
lower labial mucosa, buccal mucosa and
tongue. Based on these findings, Steven
Johnson syndrome was given as provisional
diagnosis. An array of investigations was
carried out which revealed normocytic
normochromic blood picture.
Patient was managed with injection
betamethasone, phenytoin, saline gargles,
along with antihistamine and antibiotic.
Patient was followed up 10 days later and
healing of oral lesions was reviewed (Figure
4).

Discussion
Stevens-Johnson syndrome a form of
mucocutanous disorder occurs most often in
children and young adults. Incidence ranges
from 1.2 to 6 cases per million per year (4). In
70% of SJS cases, drugs are found to be
causative agents and more than 100 such
agents have been reported. In SJS, its
necessary to take drug history carefully and
repeatedly before the causative agent can be
identified (2). In our patient one week before
the occurrence of the lesions, his drug
regimen for epilepsy was changed from
phenytoin to phenobarbitone. So, we could
postulate that phenobarbitone was the
offending drug that had caused Steven
Johnson syndrome. Ruggiero et al., reported
SJS in two children with brain tumour while
receiving
cranial
irradiation
and
anticonvulsant therapy with phenobarbital
(5). Similarly, Adeloye et al., reported two
cases of Stevens-Johnson syndrome in
patients with penetrating head wounds who
were treated with phenobarbitone (6).
Stevens-Johnson syndrome can be
preceded by a prodrome consisting of fever,
malaise, sore throat, nausea, vomiting,
arthralgias, and myalgias. This prodrome is
followed within 14 days by conjunctivitis and
by bullae on the skin and on the mucosal
membranes of the mouth, nares, pharynx,
esophagus, urethra, and vulvovaginal as well

as anal regions. Accordingly, our patient

reported of intermittent fever and stickiness
of eyes followed by typical target lesions.
Ocular complications occur in about
70% of patients with Stevens-Johnson
syndrome. Photophobia and a purulent form
of conjunctivitis may be present initially, but
corneal ulcerations and anterior uveitis can
develop. Secondary infection, corneal
opacity, and blindness can follow. Genital
adhesions resulting in dyspareunia, pain and
bleeding should be watched out (3, 7, 8),
however genital involvement was not
observed in our patient.
Erythema multiforme (EM) could
easily be mixed with SJS since both present
with rash and oral mucosal erosion. The
typical target lesions in EM have three
concentric zones: central dusky disk, middle
pale ring, outermost erythematous halo and
they are not found in SJS and TEN (9).
Several agents have been tried for the
management of this disorder. Systemic
corticosteroids are used in the early stage of
SJS and TEN. However, its use in SJS is still
controversial
but
should
not
be
recommended when extensive skin loss has
already occurred (10, 11). Thus, we prescribed
corticosteroids (Betamethasone) to our
patient as he was in the early stages of SJS.
Other
agents
include
intravenous
Immunoglobulin (12), cyclosporin A (13), and
thalidomide (14). Chlorhexidine rinses help
in maintaining good hygiene and white-soft
paraffin on the lips relieves the pain.

Conclusions
Stevens-Johnson syndrome is a
potentially fatal disorder with a strong
alliance to some medications. Physicians
must therefore be careful in prescribing the
drugs to their patients. Moreover, as this
condition involves multiple organs, it can be
best treated by early involvement of medical
specialists.

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Steven Johnson syndrome

References
1. Carrozzo M, Togliatto M, Gandolfo S.
Erythema multiforme. A heterogeneous
pathologic phenotype. Minerva Stomatol.
1999;48:217-26.
2. Brett SA, Phillips D, Lynn AW.
Intravenous immunoglobulin therapy for
Stevens-Johnson Syndrome. Southern
medical journal. 2001;94:342-3.
3. Ho H. Diagnosis and Management of
Stevens-Johnson Syndrome and Toxic
Epidermal Necrolysis. Medical Bulletin.
2008;13:17-20.
4. Habif TP. Clinical Dermatology. 3rd ed. St
Louis: Mosby; 1996. p 570-2.
5. Adeloye A, Familusi JB, Alabi GO, Odeku
LE. Stevens-Johnson syndrome following
anticonvulsant therapy in penetrating
head injury. Ghana Med J.1971;10:56-9.
6. Ruggiero A, Buonuomo PS, Maurizi P,
Cefalo MG, Corsello M, Riccardi R.
Stevens-Johnson syndrome in children
receiving phenobarbital therapy and
cranial radiotherapy. J Neurooncol.
2007;85:213-5.
7. Fritsch PO, Maldonado RR. StevensJohnson Syndrome toxic epidermal
necrolysis. In: Freedberg IM, Eisen AZ,
Wolff K. Fitzpatricks dermatology in
general medicine. 5th ed. Vol 1. New York:
McGraw-Hill; 1999. p 644-54.
8. Power WJ, Ghoraishi M, Lloves JM.
Analysis of the acute ophthalmic
manifestations
of
the
erythema
multiforme/Stevens-Johnson
syndrome/toxic epidermal necrolysis
disease
spectrum.
Ophthalmology.
1995;102:1669-76.
9. Bastuji-Garin S, Rzany B, Stern RS, Shear
NH, Naldi L, Roujeau JC. A clinical
classification of cases of toxic epidermal
necrolysis, Stevens-Johnson syndrome
and
erythema
multiforme.
Arch
Dermatol. 1993;129:92-6.
10. Fromowitz JS, Ramos-Caro FA, Flowers
FP.
Practical
guidelines
for
the
management of toxic epidermal necrolysis

and Stevens-Johnson Syndrome. Int J

Dermatol. 2007;46:1092-4.
11. Prins C, Kerdel FA, Padilla RS, Hunziker
T, Chimenti S, Viard I. Treatment of toxic
epidermal necrolysis with high-dose
intravenous
immunoglobulins:
multicenter retrospective analysis of 48
consecutive cases. Arch Dermatol.
2003;139:26-32.
12. Prins C, Kerdel FA. Treatment of toxic
epidermal necrolysis with high-dose
intravenous
immunoglobulins:
multicenter retrospective analysis of 48
consecutive cases. Arch Dermatol.
2003;139:26-32.
13. Zaki I, Patel S. Toxic epidermal necrolysis
associated with severe hypocalcaemia,
and treated with cyclosporine. Br J
Dermatol. 1995;133:337-8.
14. Wolkenstein P, Latarjet J, Roujeau JC.
Randomized comparison of thalidomide
versus placebo in toxic epidermal
necrolysis. Lancet. 1998;352:1586-9.

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