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Diagnosis, Classification, and Management of Erythema

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Diagnosis, Classification, and Management of Erythema Arch Dis Child 2000;83:347–352 347 Diagnosis, classification, and management of Arch Dis Child: first published as 10.1136/adc.83.4.347 on 1 October 2000. Downloaded from erythema multiforme and Stevens–Johnson syndrome C Léauté-Labrèze, T Lamireau, D Chawki, J Maleville, A Taïeb Abstract become widely accepted that EM and SJS, as Background—In adults, erythema multi- well as toxic epidermal necrolysis, are all part of forme (EM) is thought to be mainly a single “EM spectrum”. In both EM and SJS, related to herpes infection and Stevens– pathological changes in the earliest skin lesion Johnson syndrome (SJS) to drug reac- consist of the accumulation of mononuclear tions. cells around the superficial dermal blood Aims—To investigate this hypothesis in vessels; epidermal damage is more characteris- children, and to review our experience in tic of EM with keratinocyte necrosis leading to the management of these patients. multilocular intraepidermal blisters.5 In fact, Methods—A retrospective analysis of 77 there is little clinical resemblance between paediatric cases of EM or SJS admitted to typical EM and SJS, and recently some authors the Children’s Hospital in Bordeaux be- have proposed a reconsideration of the “spec- tween 1974 and 1998. trum” concept and a return to the original Results—Thirty five cases, inadequately description.15–17 According to these authors, the documented or misdiagnosed mostly as term EM should be restricted to acrally urticarias or non-EM drug reactions were distributed typical targets or raised oedema- excluded. Among the remaining 42 pa- tous papules. Depending on the presence or tients (14 girls and 28 boys), 22 had EM (11 absence of mucous membrane erosions the EM minor and 11 EM major), 17 had SJS, cases may be classified as EM major or EM 16 and three had isolated mucous membrane minor. The term SJS should be used for a involvement and were classified sepa- syndrome characterised by mucous membrane rately. Childhood EM was mostly related erosions and widespread blisters, often pre- to herpes infection and SJS to infectious dominant on the chest, and presenting with 17 agents, especially Mycoplasma pneumo- erythematous or purpuric macules. niae. Only two cases were firmly attrib- We have carried out a retrospective analysis uted to drugs (antibiotics). No patient of all patients under 15 years of age, hospital- died. EM and SJS sequelae were minor ised for EM or SJS over a 20 year period at the http://adc.bmj.com/ and steroids were of no overall benefit. Children’s Hospital in Bordeaux. Our aims Conclusion—In paediatric practice EM is were: (1) to classify childhood EM and SJS according to the clinical criteria of Bastuji- frequently misdiagnosed. The proposal 15 that SJS is drug related in adults does not Garin and colleagues ; (2) to study in children apply to children, and in our recruitment the hypothesis that typical EM is mainly related EM and SJS are mostly triggered by infec- to herpes simplex virus and SJS to drug reactions, as previously shown in adults17; and tious agents. The course of both diseases, on September 28, 2021 by guest. Protected copyright. even though dramatic at onset, leads to (3) to review our experience in the diagnosis low morbidity and mortality when appro- and management of children with EM. priate symptomatic treatment is given. (Arch Dis Child 2000;83:347–352) Patients and methods Three of us (CLL, DC, and JM) reviewed all Unité de Dermatologie Keywords: erythema multiforme; Stevens–Johnson the records and photographs of 77 children Pédiatrique, Hôpital syndrome admitted for EM or SJS in all paediatric wards Pellegrin-Enfants, Place Amélie of our hospital between 1974 and 1996. Raba-Leon, 33 076 Bordeaux Cedex, Erythema multiforme (EM) is an acute, CLINICAL CLASSIFICATION France self-limiting disease of the skin and mucous All cases were classified according to the C Léauté-Labrèze membranes described by Hebra in 18661;itis following criteria16: J Maleville characterised by symmetrically distributed skin : typical targets or raised oede- A Taïeb + EM minor lesions, located primarily on the extremities, matous papules acrally distributed (fig 1) Service de Pédiatrie, and by a tendency for recurrences. EM is said + EM major: as above, with involvement of Hôpital to be rare in childhood, and very few paediatric one or more mucous membranes Pellegrin-Enfants series concern EM.2–13 Most series include + SJS: widespread blisters predominant on T Lamireau adults and children, and when they concern the chest, presenting with erythematous D Chawki only children, Stevens–Johnson syndrome or purpuric macules and one or more Correspondence to: (SJS) and EM are not distinguished. In 1922 mucous membrane erosions (fig 2). Prof. Taïeb Stevens and Johnson described two children email: who had fever, conjunctivitis, stomatitis, and a CRITERIA FOR AETIOLOGICAL ATTRIBUTION [email protected] generalised exanthema with skin lesions dis- Herpes—Because herpes aetiology is question- Accepted 24 May 2000 tinct from EM14; but in the past 30 years it has able in most cases of EM, we decided to use the www.archdischild.com 348 Léauté-Labrèze, Lamireau, Chawki, Maleville, Taïeb Arch Dis Child: first published as 10.1136/adc.83.4.347 on 1 October 2000. Downloaded from Figure 1 (A) Typical targets of EM associated with raised oedematous papules on hand. (B) Typical targets of EM acrally distributed in a child with labial herpes. herpes score of Assier and colleagues.17 This French algorithm used for reporting adverse score (0–4) was established by adding the drug reactions.18 A score is calculated on the following criteria (one point each): recurrent basis of chronological and semiological criteria EM, history of recurrent herpes, recent clinical of skin manifestations; a minimum score of 3 is herpes (preceding EM within three weeks), and required to suspect drug involvement. In case a demonstration of a recent herpes simplex of drug intake during infection, the possible virus infection (virus isolation, positive immun- involvement of both causes was taken into ofluorescence, or seroconversion). EM was account. firmly attributed to herpes simplex virus infec- tion for a score of 2 or more, without any other suspected aetiology. Herpes virus infection was Results only suspected for a score of 1. Eleven cases were inadequately documented SJS was only attributed to Mycoplasma pneu- and excluded from the study. moniae (MP) when there was positive MP http://adc.bmj.com/ complement fixation titre and/or isolation of DIFFERENTIAL DIAGNOSIS OF EM AND SJS MP on throat cultures. MP was only suspected Twenty four of 66 cases were clearly not in cases of associated febrile pneumonia aVected by EM or SJS. Among these patients without bacteriological confirmation. nine had urticaria, seven had non-EM drug An infectious aetiology was suspected when a reactions (maculopapular rash in five cases and preceding illness was noticed without drug toxic pustuloderma in two cases), two children ingestion. had papular urticaria, two others acute haem- All drug ingestions during the preceding two orrhagic oedema, and two cases had varicella. on September 28, 2021 by guest. Protected copyright. weeks were recorded, and EM or SJS were Kawasaki disease and staphylococcal scalded attributed to drugs according to the oYcial skin syndrome were also noted (one case each). Figure 2 (A) SJS with skin and major mucous membrane involvement. (B) Widespead blisters of the chest in a child with SJS. www.archdischild.com Erythema multiforme and Stevens–Johnson syndrome 349 Table 1 Patients with erythema multiforme Arch Dis Child: first published as 10.1136/adc.83.4.347 on 1 October 2000. Downloaded from Drug ingestion one Duration of Steroids Recurrences Sequelae No., sex Age (y) Preceding illness week before Suspected aetiology disease Yes/no Yes/no Yes/no Erythema multiforme minor 1, M 8 months Immunisation* None Immunisation* 10 days No No No 2, M 1 Immunisation‡ None Immunisation* 10 days No No No 3, F 4 Pharyngitis Ampicillin (2) Infection or antibiotics 10 days No No No 4, M 6 None None Tuberculin 10 days No No No 5, M 9 Labial herpes None Herpes (3) 10 days No Yes No 6, F 9 Dyshidrosis Oxacillin (2) Herpes (1) or oxacillin 20 days No No No 7, M 10 None None Herpes (1)? 15 days No Yes No 8, M 11 Labial herpes None Herpes (2) 8 days Yes ? No 9, F 12 None None Herpes (2)? 15 days No Yes No 10, M 15 Labial herpes None Herpes (2) ? No ? No 11, M 15 Labial herpes None Herpes (3) 8 days No Yes No Erythema multiforme major 12, M 4 orf Acyclovir (1) orf 10 days No No No *13, M 6 Bronchoendoscopy Ampicillin (3) Ampicillin 10 days No No No *14, M 10 Tonsillitis None Streptococcus A? 15 days No No No 15, F 10 None None Herpes (1)? ? No Yes No *16, F 12 ? None Herpes (1)? ? No Yes No 17, M 13 Immunisation* None Immunisation?† ? Yes Yes No 18, M 13 Labial herpes None Herpes (3) ? No Yes No 19, M 13 Labial herpes None Herpes (2) ? No ? No *20, M 13 Labial herpes None Herpes (3) ? No Yes No 21, F 14 Tonsillitis Ampicillin (2) Infection or drugs? ? No No No Niflumic acid (2) *22, M 14 Upper respiratory tract infections None Sinusitis? 20 days Yes + Yes No thalidomide Herpes and drug scores are in brackets. *Vaccine. †Immunisation against diphtheria–tetanus–poliomyelitis. ‡Immunisation against measles–mumps–rubella. CLINICAL CLASSIFICATION Mean age was 8.7 years (range 8 months to 15 Forty two patients could be evaluated: 22 chil- years). The mean age of EM patients was dren had EM (11 EM minor and 11 EM higher than that of SJS patients (9.7 years ver- major), and 17 had SJS (tables 1, 2, and 3).
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