B.L.

Bazar 39

Stevens-Johnson Syndrome
Baby Lovella N. Bazar, M.D.*

ABSTRACT

Stevens-Johnson Syndrome (SJS) is a rare skin reaction, usually related to drugs

and associated with widespread destruction of the epidermis, characterized by mucous
membrane erosions and blisters on limited areas of the skin, which is less than 10% of
the total body surface area. The incidence of SJS, along with Toxic Epidermal Necrolysis
(TEN), a closely related disease is approximately 2 cases per million persons per year and
the overall mortality rate is 20%-25% (Gueudry, et.al). It is an acute systemic disease
involving eruptions of the skin and mucous membranes that predispose patients to the life-
threatening complications of sepsis which can be fatal. The patient with severe involvement
often requires intensive multispecialty care. We present a 5-year old healthy female child
who initially developed conjunctivitis and erythema of lips and buccal mucosa which later
on progressed to purulent conjunctivitis, hemorrhagic lips and erythematous macules that
rapidly and variably developed into central necrosis forming vesicles, bullae, and areas of
denudation on the face, trunk and extremities.
This case aims to familiarize us with the most common presenting signs and symptoms
of this condition, its possible complications and plan of management.
*Resident Physician Trainee I,
Department of Pediatrics
Best Poster Winner, 29th Resident Physician’s
Case Report Contest, March 5, 2010,
Tanchoco Auditorium

INTRODUCTION
This case is being presented to make us aware that
in a patient presenting with seemingly benign signs and
symptoms such as fever and conjunctivitis, there is the
possibility of developing a life threatening condition such as
Stevens-Johnson Syndrome (SJS), a severe form of erythema
multiforme, also known as erythema multiforme major.
CASE REPORT
A 5-year old female child, Filipino, Catholic residing at
Caloocan City was brought in and admitted for the 1st time
at this institution due to conjunctivitis and vesicular lesions.
She was otherwise healthy with no previous history of illnesses
prior to admission.
Four days prior, patient was noted to have fever assessed
by touch and subconjunctival, non-pruritic erythema of
left eye. No consultation was done, no medications were
taken. Condition persisted until 3 days PTA when both eyes
were noted to have yellowish discharge and erythematous
macular rashes were also noted on her face. Consult to a
private doctor was done where patient was prescribed with
Chloramphenicol (28mkd), Diphenhydramine (3mkd) and
Paracetamol (10mkd) which the patient took for 1 day. 2 days
PTA, the rash developed into vesiculo-bullous lesions spread
on her face, trunk and extremities. Consult was again done,
Chloramphenicol was shifted to Cefalexin (42mkd). 1 day
PTA, patient started to have episodes of vomiting and was
noted to have poor appetite and body weakness. Few hours
PTA, patient become progressively listless and ill-appearing.
At this time she was also noted to have dry cracked lips with
swelling, erosions and bleeding. This prompted consult at the
ER where she was subsequently admitted.
Physical examination revealed a conscious, ill-looking
young child in no respiratory distress with a BP of 90/60mmHg,
cardiac rate of 108 beats/min and respiratory rate of 26
cycles/min. Skin was warm; with purpuric macules spread
over her entire body. Head and neck showed hyperemic
conjunctiva with purulent eye discharge; with swelling and
hemorrhagic crusting of the lips; tonsils were hyperemic.
Chest had symmetrical expansion, no retractions, lungs
had clear breath sounds. She had adynamic precordium,
cardiac rate was normal with regular rhythm, no murmurs.

Stevens-Johnson Syndrome
40 Philippine Scientific Journal Vol. 43. No. 2

Abdomen was flat, soft, with normoactive bowel sounds, no
hepatomegaly, no tenderness elicited on palpation. Extremities
were grossly normal, with full, equal pulses. External female
genitalia was normal looking but with same purpuric macular
lesions. Patient had no previous hospitalization; no history
of asthma and allergies to foods/drugs. Her birth history was
unremarkable and developmental milestones at par with age;
immunization was completed at the Health Center.
An initial impression of Steven-Johnson Syndrome (SJS)
was made. On admission patient was started on intravenous
fluid and normal saline oaks for the lips were administered
as supportive care. She was also started on Hydrocortisone
intravenously at 5mkd, Famotidine 1mkd and oral Paracetamol
at 11mkd. Miconazole was also applied on her oral sores. Chest
x-ray done was normal. With the development of conjunctivitis
bilateral, she was given Erythromycin ophthalmic ointment.
2nd hospital day (day 5 of illness) patient was afebrile but
irritable; purpuric macules became bulllous and patient
complained of pruritus as well as pain on the sites of the
lesions. Patient was given Loratadine at 0.4mkd and Mupirocin
ointment. On the 3rd hospital day (day 6 illness), patient still
complained of pruritus hence was given Diphenhydramine
and Hydroxyzine. On the 4th hospital (day 7 of illness), target
lesions were noted on the palms and soles.
Continuous supportive measures rendered resulted in
gradual improvement and the patient was discharged on the
6th hospital day (day 9 of illness), when the patient was able
to tolerate oral fluids and nutrition.
erythrocyte sedimentation rate, and occasionally, increased
transaminase levels and decreased serum albumin values.
The pathogenesis of SJS is not well understood.
Speculations on the possible causes includes drugs such as
anti-infectives (sulfonamides, trimethoprim-sulfamethoxazole,
aminopenicillins, quinolones, cephalosporins, and
tetrayclines); oxicam NSAIDs, aromatic anticonvulsant drugs
(phenobarbital, phenytoin, carbamazapine), and allopurinol
(Jean-Claude Roujeau, et.al). However, indirect evidence, such
as demonstration of circulating immune complexes, deposits
of IgM and C3 in the dermal vessels, and demonstration of
cytotoxic T lymphocytes in association with keratinocytes, also
suggest the role of both humoral and cell mediated immunity
in its pathogenesis (Asha Moudgil, MD).
Management is supportive and symptomatic. Potentially
offending drugs should be discontinued as soon as possible.
The use of steroids is controversial and nowadays, intravenous
immunoglobulins (IVIG) is widely being studied
ILLUSTRATION

DISCUSSION

We are presented with a case of a 5-year old female child

who was apparently healthy with no known risk factors for
developing a condition such as SJS.
Stevens-Johnson Syndrome (SJS) is a potentially
life-threatening, mucocutaneous disorder characterized
by epidermal detachment with mucosal involvement. It
is a cutaneous lesion that generally consists initially of
erythematous macules that rapidly and variably develop central
necrosis to form vesicles, bullae, and areas of denudation on the
face, trunk, and extremities. (Nelson’s Textbook of Pediatrics).
The skin lesions are typically widespread and is accompanied
by involvement of two or more mucosal surfaces, namely the
eyes, oral cavity, upper airway or esophagus, gastrointestinal
tract, or anogenital mucosa. A burning sensation, edema,
and erythema of the lips and buccal mucosa are often the
presenting signs, followed by development of bullae, ulceration
and hemorrhagic crusting. Lesions may be preceded by a flu-
like upper respiratory illness. Pain from mucosal ulceration is
often severe, but skin tenderness is minimal to absent (Nelson’s
Textbook of Pediatrics). Of these symptoms being described,
many were noted to be present on our patient. Nonspecific
laboratory abnormalities in SJS is leukocytosis, elevated
 B.L. Bazar 41

CONCLUSION

Clinical manifestations of SJS includes hemorrhagic oral

mucosal and conjunctival lesions that cause severe discomfort
and disability. The clinical course is usually prolonged and
illness may last up to six weeks. We are presented here with a
case of a 5-year old female child who developed an acute onset
of purulent conjunctivitis and purpuric macular rash which
later on became vesico-bullous affecting her face, extremities,
trunk and ano-genital areas.
Several complications may arise if identification of
such condition is delayed. Among these complications,
ophthalmologic is the most common and consultation is
mandatory because ocular sequelae such as corneal scarring can
lead to visual loss. Other complications include permanent
skin damage and scarring, secondary skin infection, systemic
infection and severe loss of body fluids resulting in shock.
It may also affect systemic organ and cause Pneumonitis,
Myocarditis, Enterocolitis, Nephritis and Hepatitis
The most probable cause for this case could either be viral,
immune mediated or idiopathic. Patient may be admitted at
ICU or burn unit and ophthalmologic consultation should be
obtained. Once discharged, sun exposure should be avoided
and advised to use sunblock lotions. Precaution should also
be done when taking drugs.

REFERENCES

1. Kliegman, Behrman, Jenson, Stanton: Nelson Textbook

of Pediatrics 18th Edition pp.990-993; 2685-2690
2. Jean-Claude Roujeau et.al., Medication Use and the
Risk of Stevens-Johnson Syndrome or Toxic Epidermal
Necrolysis, The New England Journal of Medicine,
Vol.333 No.24, Dec.1995, pp1600-1606
3. Asha Moudgil, MD et.al: Treatment of Steven-Johnson
Syndrome with Pooled Human Intravenous Immune
Globulin. Clinical Pediatrics January 1995, pp48-51

Stevens-Johnson Syndrome
42 Philippine Scientific Journal Vol. 43. No. 2

4. Julie Gueudry, MD; Jean-Claude Roujeau, MD; Michel

Binaghi, MD: Gisele Soubrane, MD, PhD; Marc Muraine
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