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Archives de pédiatrie
Volume 19, n° 11
pages 1182-1186 (novembre 2012)
Doi : 10.1016/j.arcped.2012.08.021
Received : 22 February 2012 ;  accepted : 17 August 2012
Une lèpre d’évolution atypique
Leprosy with an unusual course
 

A. Desenfants a, d, , E. Huguon a, Y. Polfrit a, M. Crouzat b, V. Rouleau c, V. Chassot a, L. Besson-Leaud a, I. Missotte a
a Service de pédiatrie, hôpital Magenta, centre hospitalier territorial de Nouvelle-Calédonie, BP J5, 98849 Nouméa cedex, France 
b Service de dermatologie, hôpital Gaston-Bourret, centre hospitalier territorial de Nouvelle-Calédonie, BP J5, 98849 Nouméa cedex, France 
c Laboratoire d’anatomocytopathologie, institut Pasteur, centre hospitalier territorial de Nouvelle-Calédonie, BP J5, 98849 Nouméa cedex, France 
d Faculté de médecine de Montpellier, université Montpellier 1, 2, rue École-de-médecine, 34060 Montpellier cedex 2, France 

Auteur correspondant.
Résumé

La maladie de Hansen ou lèpre est une affection rare et peu connue dans les pays développés. Sa prévalence en Nouvelle-Calédonie est stable ces dernières années autour de 0,35 pour 10 000 habitants. Nous rapportons le cas d’une enfant de 11ans qui avait présenté une lèpre lépromateuse compliquée d’une réaction de type 2 avec des signes biologiques rappelant un syndrome d’activation macrophagique. Après un rappel sur la maladie de Hansen et ses complications, nous discutons des différentes hypothèses pouvant expliquer l’évolution clinique et biologique, et les traitements qui ont permis une évolution favorable de la maladie.

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Summary

In developed countries, Hansen disease, or leprosy, is a rare and little-known disease. Over the last few years, its prevalence in New Caledonia has remained stable (0.35 per 10,000inhabitants). We report the case of an 11-year-old child who presented lepromatous leprosy complicated by a type 2 reaction. Despite appropriate treatment, the course was unusual with fever lasting a few weeks associated with asthenia, weight loss, and biological perturbations such as inflammatory syndrome, anemia, and hyperferritinemia. After a brief review of Hansen disease and its complications, we discuss the different hypotheses that can explain the clinical and biological progression of our patient (hemolytic anemia secondary to dapsone, type 2 reaction, and aspects of hemophagocytic syndrome) and describe therapeutic management, which led to a good outcome.

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