CLINICAL REPORT
489 Advances in dermatology and venereology ActaDV Acta Dermato-Venereologica ActaDV
Paediatric Erythema Multiforme: Epidemiological, Clinical and Laboratory Characteristics
Yael SIEDNER-WEINTRAUB 1, Itai GROSS 1, Almog DAVID 2, Shimon REIF 1 and Vered MOLHO-PESSACH 3
1
Pediatric Division and 3 Department of Dermatology, Hadassah-Hebrew University Medical Center, and 2 Faculty of Medicine, Hebrew University, Ein Kerem, Jerusalem, Israel
Erythema multiforme( EM) is an immune-mediated reaction presenting as acrofacial target lesions. Most studies utilize the outdated classification, which includes EM, Stevens – Johnson syndrome and toxic epidermal necrolysis as related entities. We describe here epidemiological, aetiological, clinical, laboratory and treatment characteristics of paediatric EM. This is a retrospective single-centre study, performed between 2000 and 2013. Of 119 children given a diagnosis of EM, only 30 met clinical criteria and were included in this study. Most misdiagnosed cases were non-specific eruptions and urticaria multiforme. Mean age was 11.3 years. Fifty percent had mucosal involvement. An aetiology was observed in half of the patients. Seventy percent of patients were admitted to hospital, 46.7 % were treated with systemic steroids. Sixteen percent had recurrent EM. The most common identified infectious agent associated with EM in this study was Mycoplasma pneumonia and the cases associated with this infection may represent the recent entity, mycoplasma-induced rash and mucositis. Association with herpes simplex virus was not observed. Despite being a benign, self-limiting condition, children were over-treated in terms of hospitalization and therapy.
Key words: erythema multiforme; children; erythema multiforme minor; erythema multiforme major.
Accepted Nov 8, 2016; Epub ahead of print Nov 10, 2016 Acta Derm Venereol 2017; 97: 489 – 492.
Corr: Vered Molho-Pessach, Department of Dermatology, Hadassah-Hebrew University Medical Center, Kiryat Hadassah, POB 12000, Jerusalem, 9112001, Israel. E-mail: rverem @ hadassah. org. il
Erythema multiforme( EM) is a self-limiting, immunemediated reaction, which manifests with a typical mucocutaneous eruption. The characteristic skin lesion of EM is the target lesion, which measures < 3 cm in diameter, has a round shape and well-defined border, and consists of 3 distinct zones; 2 concentric rings of colour change surrounding a central circular zone, which tends to have a dusky appearance, manifesting damage to the epidermis in the form of a bulla or crust. Atypical lesions with only 2 different zones and / or a poorly defined border may also exist( 1). The rash tends to involve acrofacial sites. It may be confined to the skin and is then termed EM minor, or it may involve mucosal membranes, and is then termed EM major( 2, 3).
First described by von Hebra in 1860, EM had long been considered as part of a spectrum of diseases, including EM minor, EM major, Stevens – Johnson syndrome( SJS) and toxic epidermal necrolysis( TEN)( 4). These entities were thought to represent an immune reaction to one of several different aetiologies, which could be limited to the skin, extend to involve mucosal membranes, or present as a systemic inflammatory state, with significant morbidity and mortality( 5). In 1993 Bastuji-Garin et al.( 6) proposed a novel classification: EM as a different entity from SJS / TEN, with its own aetiology, pathophysiology and clinical course. EM has several different aetiologies; the most common cause is infectious, encompassing approximately 90 % of cases( 7). Herpes simplex virus( HSV) is estimated to be the cause of EM in approximately 70 % of patients( 8) and preceding herpes labialis is noted in 50 % of subjects with EM. In these cases, EM tends to recur several times( 9). Mycoplasma pneumoniae is also a prominent pathogen, which may also be associated with SJS( 10, 11). Various other aetiologies have been associated with EM( drugs, auto-immune disorders, malignancy, radiation, etc.), but are considered of far less importance( 3).
EM affects mostly young adults, but can also appear in children( 4). Most of the previous studies describing paediatric EM refer to the outdated classification of EM as part of the EM – SJS – TEN spectrum( 12 – 15).
The aim of this study was to characterize paediatric EM as a separate entity, and to describe epidemiological, aetiological, clinical, laboratory and treatment characteristics in this population.
METHODS
This retrospective epidemiological descriptive study was conducted at the Hadassah Hebrew University Medical Center, a tertiary hospital in Jerusalem, Israel. Data on all cases of children( age range 0 – 18 years) with a clinical diagnosis of EM( ICD9 695.11, 695.12, 695.10.09, 695.19) who were examined at our emergency, paediatric
Table I. Criteria for diagnosis of erythema multiforme( EM)( from Bastuji-Garin et al.( 6))
Criteria for EM
Typical target lesions: individual lesions less than 3 cm in diameter with a regular round shape, well defined border, and at least 3 different zones, i. e. 2 concentric rings around a central disk. One ring consisted of palpable oedema, paler than the centre disk
Raised atypical targets: round, oedematous, palpable lesions, reminiscent of EM, but with only 2 zones and / or a poorly defined border
Less than 10 % body surface area
This is an open access article under the CC BY-NC license. www. medicaljournals. se / acta Journal Compilation © 2017 Acta Dermato-Venereologica. doi: 10.2340 / 00015555-2569 Acta Derm Venereol 2017; 97: 489 – 492