Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
3182053 | Actas Dermo-Sifiliográficas | 2006 | 5 Pages |
ResumenPresentamos el caso de una niña de 10 semanas de edad que presentaba lesiones papulosas eritemato-amarillentas desde el nacimiento con diagnóstico de histiocitosis de células de Langerhans, que se acompañaba de lesiones líticas en cráneo y afectación hepática. Tras varios meses de tratamiento con prednisona y vinblastina, con mejoría cutánea y sistémica, aparecieron varias lesiones papulosas eritematoamarillentas redondeadas en mejilla derecha; la biopsia mostró un infiltrado histiocitario con positividad para CD68 y tinción negativa para S100 y CD1a, con diagnóstico final de xantogranuloma juvenil. Dicha asociación ha sido previamente descrita en la literatura en pocos casos; aunque se han insinuado varias hipótesis, la relación causal entre ambas entidades aún no ha sido demostrada.
We present the case of a 10-week-old girl who had erythematous papules with a yellowish hue from birth with diagnosis of Langerhans cell histiocytosis, that was accompanied by a lytic lesion in the skull and hepatic involvement. After several months of treatment with prednisone and vinblastine with skin and systemic improvement, several rounded erythematous papules with a yellowish hue appeared in the in right cheek. The biopsy showed a histiocytic infiltrate with positivity for CD68 and negative staining for S100 and CD1a, with a final diagnosis of juvenile xanthogranuloma. This association has been previously described in the literature in few cases. Although several hypotheses have been suggested, the causal relationship between both entities has still not been demonstrated.