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Kasuistik Allergologie, Jahrgang 34, Nr. 5/2011, S.

264–267

Case report
Stevens-Johnson syndrome/toxic epidermal
necrolysis in a child
M. Tichy1, I. Karlova1, M. Tichy2, J. Urbanek1 and D. Ditrichova1

1Department of Dermatology and Venereology, and 2Department of Pathology,


University Hospital, Faculty of Medicine and Dentistry, Palacky University Olomouc,
Czech Republic

Stevens-Johnson syndrome/TEN in a child


Schlüsselwörter Stevens-Johnson-Syndrom/toxische Key points
Stevens-Johnson-Syn- epidermale Nekrolyse bei einem Kind
drom/Toxische epider- Die vorliegende Kasuistik beschreibt den – Stevens-Johnson syndrome/toxic epider-
male Nekrolyse – Ätiolo- Fall eines 9-jährigen Jungen mit schwerer
gie – Infektionen –
mal necrolysis (SJS/TEN) belongs among
bullöser Hautreaktion. Der Junge war auf- the most serious and life-threatening der-
Medikamente
grund einer Atemwegsinfektion mit Penizil-
lin behandelt worden und hatte daraufhin ein matological diseases of childhood.
Key words disseminiertes makulöses Exanthem mit eini- – This is characterized by bullous forma-
Stevens-Johnson syn- tions that cover 10 – 30% of the skin sur-
gen atypischen Zielläsionen an Rumpf und
drome/toxic epidermal
necrolysis – etiology –
Extremitäten entwickelt. Später traten Fieber, face and extensive mucosal involvement.
infections – drugs
Krankheitsgefühl und Bullae auf, die etwa – Drug-induced etiology is most common
25 – 30% der Hautoberfläche bedeckten. Die
in adults, while infections are important
klinische Manifestation, die histopathologi-
sche Untersuchung, die direkte Immunfluo- triggers in children.
reszenz sowie die serologischen Tests stütz-
ten die Diagnose Stevens-Johnson-Syndrom/
toxische epidermale Nekrolyse (SJS/TEN).
Im Folgenden werden die Merkmale der kli-
Introduction
nischen Subtypen des Erythema multiforme
(EM) und der schweren bullösen Reaktionen, Erythema multiforme (EM) is a polyetio-
ihre Ätiopathogenese sowie auch die thera- logic disease characterized by various often
peutische Vorgehensweise und die Indikatio- overlapping symptoms [2]. Five different
nen für systemische Kortikosteroidverabrei- variants of the disease are differentiated by
chung diskutiert. popular convention although their respective
symptoms often overlap (Table 1).
Instructive case: Stevens-Johnson syn- Severe bullous skin reactions are among
drome/toxic epidermal necrolysis in a the most serious and life-threatening derma-
child
tological diseases of childhood. We report a
The authors describe the case of a 9-year-
old boy with a severe bullous skin reaction. case of SJS/TEN, which developed after re-
Initially, disseminated macular exanthema spiratory infection treated with penicillin.
with a few atypical target lesions on the trunk
and extremities followed respiratory infec-
tion treated with penicillin. Later, fever, mal-
aise and progression to blisters that cover Case report
about 25 – 30% of the skin surface developed.
The clinical manifestation, histopathological A 9-year-old boy was initially treated by a
examination, direct immunofluorescence and pediatrician for tonsillitis with orally admin-
serological tests were pivotal for diagnosis of istered penicillin. After 3 days of treatment
SJS/TEN. The general characteristics of the the patient began to develop shortness of
clinical subtypes of EM and severe blistering
breath concurrently with a macular exan-
reactions and their etiopathogenesis are dis-
cussed along with therapeutic procedure thema over his entire body’s surface area that
© 2011
Dustri-Verlag Dr. Karl Feistle
including indications for systemic cortico- focally progressed to form blisters requiring
ISSN 0344-5062 steroid administration. hospitalization of the patient in the pediatric
Stevens-Johnson syndrome/toxic epidermal necrolysis in a child 265

Table 1. Clinical types of erythema multiforme.

criteria EM minor form EM major form SJS SJS/TEN TEN

target lesions + + – – –
macules – – + + + (or large erythema)
blisters, erosions – < 10% < 10% 10 – 30% > 30%
mucosal involvement – + + extensive extensive
distribution extremities mainly extremities diffuse diffuse widespread

EM: erythema multiforme; SJS: Stevens-Johnson syndrome; TEN: toxic epidermal necrolysis.

Figure 1, 2. Formation of multiple blisters and secondary erosions on the skin and mucose membranes of
the mouth and genitalia.

clinic (Figure 1, 2). The development of blis- The histopathologic examination of the
ters was associated with malaise and fever. skin was pivotal for the diagnosis of EM.
Bullous formations occured mainly on the Characteristic findings were found, and they
face, trunk and lower extremities and covered included significant vacuolar dystrophy rang-
approximatelly 25 – 30% of the skin surface. ing to balloon degeneration of the basal cell
Mucosal lesions on the lips and genitalia were layer of the epidermis with the formation of
eroded. Based on the clinical presentation and intraepidermal blisters, sometimes penetrat-
the course of disease, the initial differential ing subepidermally through the basal layer.
diagnostic workup included mainly the se- Apoptotic keratinocytes were present in the
vere form of erythema multiforme and con- basal layer, and in a dispersed fashion in the
sideration was also given to the diagnosis of upper layers of the epidermis (Figure 3). The
herpetic eczema due to the patients history of upper dermis was edematous, and a not so in-
dermorespiratory syndrome. The boy was tense pericapillary lymphatic infiltrate was
treated with acyclovir, hydrocortisone, and present near the necrobiotically altered kera-
the ATB therapy was changed from penicillin tinocytes at the dermoepidermal junction.
to clarithromycin. Melanophages were found to be copiously
A number of additional tests were per- dispersed in the corium.
formed for the purpose of diagnostic verifica- Direct immunofluorescence resulted in
tion. The blood count and basic biochemical non-specific findings with fibrinogen and a
and urinary tests were normal, and the labora- small amount of all classes of immunoglo-
tory markers of inflammation (ESR, CRP) bulins and C3 in blister content.
were negative. The performed serological In correlation with the clinical presenta-
tests for infectious mononucleosis and herpes tion, which was dominated by disseminated
simplex only showed anamnestic antibody eruptions of secondary blister erosion cover-
titres whereas the presence of serological evi- ing about 25 – 30% of the skin surface on the
dence for active mycoplasmal and chlamydial face, trunk, genitalia and lower extremities
infections was found. along with extensive erosions on the lips and
a mild conjunctivitis and absence of typical
Tichy, Karlova, Tichy, Urbanek and Ditrichova 266

another type of drug-induced reaction, the


high-risk medicaments of short-term utiliza-
tion are trimethoprim-sulfamethoxazole, sul-
fonamides, aminopenicillins and quinolones;
and the high-risk medicaments of long-term
utilization consist particularly of the anti-epi-
leptic drugs carbamazepine, phenytoin and
phenobarbital [2, 3]. The precise mechanism
of pathogenesis of these reactions is un-
known, but it is generally believed that it is a
Figure 3. HE 100 × – Significant vacuolar dystro- type II cytotoxic hypersensitivity.
phy ranging to balloon degeneration of the basal cell Infectious etiology is more common in
layer of the epidermis with the formation of intraepi-
mild presentations of the minor and major
dermal blisters, focally penetrating subepidermally
through the basal layer. Apoptotic keratinocytes in forms of EM. The most common proven asso-
the basal layer, dispersed melanophages in edema- ciations with infection have been demon-
tous dermis. strated to be with herpes simplex and Myco-
plasma pneumoniae; less commonly with
other coincidental infections such as hepatitis
target lesions, the condition was diagnosed as B and sporadically with some others [2, 6, 8,
SJS/TEN. Antiviral therapy was discontin- 9]. Infectious etiopathogenesis of SJS and
ued and the patients treatment was gradually SJS/TEN is more likely in children than in
altered from parenteral hydrocortisone to oral adults [5, 6]. The studies of some authors ac-
prednisone with gradual dose reduction until tually confirm that infectious etiologies are
discontinuation in less than 4 weeks. Given the causal factors of SJS or SJS/TEN in the
the serological findings of active mycoplas- majority of observed children and that drug
mal and chlamydial infection, treatment with etiology is considered to be rather rare in pe-
clarithromycin was prolonged for the dura- diatric patients [5]. It is not commonly possi-
tion of two extra weeks. The therapeutic plan ble to clearly specify the etiopathogenesis of
was supplemented with antihistamines and EM in complicated cases where both the pres-
topical corticosteroid treatment combined ence of an infectious agent and a positive
with antibiotics. This method of treatment pharmacological history of a high-risk medi-
gradually led to the complete regression of cation is found to be simultaneous. The cor-
the objective and subjective symptoms of rect management is then to eliminate both of
SJS/TEN. the possible causes by the discontinuation of
the suspected inappropriate medicament and
treatment of the infection concurrently with
Discussion the introduction of more complex therapy at
burn centers in cases of TEN [2, 3, 6].
The clinical symptoms of the severe forms The most discussed topic pertaining to the
of skin blistering reactions can overlap. The treatment of severe forms of EM is the admin-
classification of the individual subtypes (SJS, istration of systemic corticosteroid therapy
SJS/TEN, TEN) is based primarily on the ex- and this holds true especially when taking
tent of skin blistering and mucosal involve- into consideration cases of TEN. One group
ment [1, 2, 3, 4, 9]. In addition to patient his- of experts considers treatment with cortico-
tory and clinical presentation, histopatho- steroids as beneficial in severe cases that are
logical examination with the above described clearly caused by drugs. This notion is sup-
characteristics is beneficial for the determina- ported by a number of specialists such as the
tion of diagnosis. Examination by direct majority of ophthalmologists consider it to be
immunofluorescence does not result in spe- a crucial step in the prevention of eye inflam-
cific findings. While searching for the cause mation and resultant severe scarring of the
of bullous reactions, it is particularly essential cornea [2]. Opponents of the issue of treat-
to consider drug-induced etiology. Although ment with systemic corticosteroids argue of
a number of pharmaceuticals can theoreti- its immunosuppressive effect and possible
cally cause severe bullous skin reactions or consequent potentiation of associated infec-
Stevens-Johnson syndrome/toxic epidermal necrolysis in a child 267

tions. Systemic corticosteroid therapy in chil- B virus infection. Clin Exp Dermatol. 2006; 31:
137-138.
dren is viewed by the majority of health pro-
[8] Schalock PC, Brennick JB, Dinulos JG. Myco-
fessionals more skeptically, given the proven plasma pneumoniae infection associated with
infectious etiology of most cases of SJS and bullous erythema multiforme. J Am Acad Der-
SJS/TEN [6]. Based on our experience, we matol. 2005; 52: 705-706.
[9] Williams PM, Conklin RJ. Erythema multiforme: a
consider the administration of systemic corti-
review and contrast from Stevens-Johnson syn-
costeroids in cases of suspected drug-induced drome/toxic epidermal necrolysis. Dent Clin
etiopathogenesis to be well-founded in the North Am. 2005; 49: 67-76.
early stages of SJS, SJS/TEN and TEN in
M. Tichy, MD, PhD
both adults and children. Stating this, by the
Department of Dermatology and Venereology
description of our case we document a favor- University Hospital
able course of illness followed by induced re- Faculty of Medicine and Dentistry
mission accomplished with the use of the Palacky University Olomouc
I.P. Pavlova 6
above mentioned therapeutic management. 775 20 Olomouc, Czech Republic
Other treatment options mentioned in re- e-mail: tichy.martin.jun@fnol.cz
ported cases of successfully treated severe
forms of EM are for example intravenous
immunoglobulins (IVIG), dapsone, inter-
feron alfa, or cyclosporine [2, 3].
Depending on the severity and extent of
the disease it is often essential to establish a
working cooperation between pediatrician,
dermatologist and burn specialist.

Conflicts of interest
All authors declare that they have no con-
flict of interest.

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