International Journal of Pediatric Otorhinolaryngology 77 (2013) 284–286

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International Journal of Pediatric Otorhinolaryngology

journal homepage: www.elsevier.com/locate/ijporl

Case report

Stevens–Johnson syndrome: An oral viewpoint

Sergio Sargenti Neto a,b, Luiz Fernando Barbosa de Paulo a,*, Roberta Rezende Rosa a,
Antônio Francisco Durighetto a
a
Program of Specific Care of Oral Diseases, Stomatology Diagnosis Unit, Faculty of Dentistry, Federal University of Uberlândia, MG, Brazil
b
Post-Graduation Course in Pathology, Institute of Biological Sciences and Natural, Federal University of Triângulo Mineiro, Uberaba, MG, Brazil

A R T I C L E I N F O A B S T R A C T

Article history: The Stevens–Johnson syndrome (SJS) is a severe variant of erythema multiforme (EM) that occasionally
Received 26 August 2012 can rise to systemic upset and possibly compromise life. In this report, we described important oral and
Received in revised form 22 October 2012 dermatologic aspects of a case of SJS triggered by Mycoplasma pneumoniae infection.
Accepted 22 October 2012
ß 2012 Elsevier Ireland Ltd. All rights reserved.
Available online 11 November 2012

Keywords:
Erythema multiforme
Mouth
Stevens–Johnson syndrome
Infection
Mycoplasma pneumonia
Mucosal membranes

1. Introduction observed in others immunological disorders [1,2,5]. There are no

The Stevens–Johnson syndrome (SJS) is a variant of erythema
multiforme, a rare acute mucocutaneous condition caused by a
hypersensitivity reaction [1]. SJS usually involves two or more
mucous membranes with more variable skin involvement, it can
involve internal organs, and there is a 10% mortality rate for
patients with extensive disease [1–4].
SJS has been reported to be triggered by numerous agents,
particularly viruses and variety of other infectious agents, immune
conditions, non-infectious agents, such as food additives or
chemicals (benzoates and nitrobenzene) and drugs [1–9]. Not-
withstanding of this, in most patients an association between
presence of SJS and cause is unclear, although there is a consensus
in the literature that all the patients harbored SJS developed an
immunological hypersensitivity reaction after antigenic challenge
[1,2,5].
The diagnosis of SJS is often difficult because the clinical picture
mimics different oral inflammatory and vesiculobullous diseases
[1–3]. The microscopic examination of SJS is nonspecific and
different pathologic aspects commonly seen in this entity may be
* Corresponding author at: Program of Specific Care of Oral Diseases – PROCEDE,
Stomatology Diagnosis Unit, Federal University of Uberlândia, Rua Acre 94138450-
319, Uberlândia, Minas Gerais, Brazil. Tel.: +55 34 32324786.
E-mail address: luizfbpaulo@gmail.com (L.F. Barbosa de Paulo).
standardized guidelines for treatment of SJS. Recognition and
prompt discontinuation of the offending agent is necessary to
control the disease [1,2].
2. Case report
A 6-year-old boy presented with high fever, dysphagia, severe
mouth pain, photophobia, and cough. His symptoms began in the
previous 4 days, and no medical interventions were performed
until that moment. Physical exam showed swelling and blistering
in the mouth, bilateral conjunctivitis, and spreading concentric
skin blisters/ulcer lesions, which began on the neck and spread to
the trunk, extremities (Fig. 1) and genital area. The lips vermilion
was covered with pseudomembranous ulcers, the tongue also
presented ulcers in the border and all lesions in touch caused pain
and bleeding (Fig. 2). The gums presented no evidence of lesion
(Fig. 3). Lung auscultation revealed bilateral wheezing and fine
end-inspiratory crepitations. Laboratory investigations documen-
ted a seroconversion of mycoplasma antibodies. The chest
radiograph showed an atypical pneumonia. The diagnosis of
Stevens–Johnson syndrome due to a Mycoplasma pneumoniae
infection was made. Therapy consisted of clarithromycin (15 mg/
kg per day) during 6 days, combined with an appropriate
symptomatic treatment. Within 1 week, the mucous membrane
lesions resolved completely. A follow-up assessment 4 weeks after
discharge was normal.

0165-5876/$ – see front matter ß 2012 Elsevier Ireland Ltd. All rights reserved.
http://dx.doi.org/10.1016/j.ijporl.2012.10.019
 S. Sargenti Neto et al. / International Journal of Pediatric Otorhinolaryngology 77 (2013) 284–286
Fig. 1. The concentric blisters/ulcers lesions (target’s lesions or bull’s eye lesion) in
the EM/SJS. The skin lesions are not necessary for a diagnosis in EM/SJS.
Fig. 2. The typical characteristic of crusted lips in the patient with EM/SJS.
3. Discussion
The nomenclature of different manifestations of erythema
multiforme (EM) is unclear [1–3]. The classification in this report
was based in the number of affected mucous membranes, one
mucosa only in the EM minor and two or more mucosa in EM
major/Stevens–Johnson syndrome (SJS) [4].
The first complaint generally is related with flu-like symptoms,
lesions that affect the mouth, eyes, skin and/or genitals [1,2,5].
Although, the clinical appearance can be difficult to differentiate
from other vesiculobullous disorders such as pemphigus,
285
Fig. 3. The no compromise of gums in the patient with EM/SJS (A – superior and
B – inferior).
pemphigoid, viral stomatitis, Behçet’s syndrome or Crohn’s
disease [2,6].
The blistering and ulcerations in lips created the aspect of
crusted lips, the most characteristic aspect in SJS [4,6]. The tongue,
cheeks and oral vestibule could be affected by blisters and become
extensive ulcers [1]. But, the gingival involvement is rare and when
it is present the commitment is extremely reduced in comparison
with mucosal lesions [7]. This is a strong evidence for the clinical
diagnosis in SJS in our view.
The complementary clinical aspect included ocular lesions,
genital lesions (similar to the oral lesions) and skin lesions that
show a specific organization in concentric blisters/ulcer lesions
(target’s form or bull’s eye form) [1–3]. There are no specific
diagnostic tests for EM/SJS, and so the diagnosis is usually
supported by the medical history and clinical aspects [1]. For
the diagnosis of Mycoplasma pneumonia infection, the frequently
used serological methods, often allow a diagnosis in late phases of
disease. However, it was recently shown that PCR is superior to
serology for diagnosis during the early phase of infection and it
represents an interesting alternative [8].
The management must be based in the removal or treat of any
precipitants. Macrolides have been the treatment of choice for
pediatric M. pneumoniae infections. In this case the use of
clarithromycin provides a resolution of disease. However, it was
recently reported that widespread use of macrolide antibiotics
increasing the incidence of macrolide resistant strains [10].
To control the signs and symptoms normally is used analgesics,
anti-inflammatory and anesthetic, especially in those cases of
severe and painful oral lesions [2,3]. The importance of adequate
fluid intake should be emphasized. Corticosteroids are commonly
used in the EM, but in patients with history of infectious diseases it
could risk the patient [6,7]. The use of intravenous immunoglobu-
lin also has been described as an effective treatment for SJS,
however, there is insufficient evidence to recommend the routine
use of this technique [11].
Conflicts of interest
This study had no financial sponsorship and we have no
potential conflicts of interest to declare.
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