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Neonatal systemic juvenile Xanthogranuloma with Hydrops diagnosed by Purpura skin biopsy: a case report and literature review.
Uehara, Yohji; Wada, Yuka Sano; Iwasaki, Yuka; Yoneda, Kota; Ikuta, Yasuhisa; Amari, Shoichiro; Maruyama, Hidehiko; Tsukamoto, Keiko; Isayama, Tetsuya; Sakamoto, Kenichi; Shioda, Yoko; Miyazaki, Osamu; Irie, Rie; Yoshioka, Takako; Mochimaru, Naoko; Yoshida, Kazue; Ito, Yushi.
Afiliación
  • Uehara Y; Department of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan.
  • Wada YS; Department of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan. wada-y@ncchd.go.jp.
  • Iwasaki Y; Department of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan.
  • Yoneda K; Department of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan.
  • Ikuta Y; Department of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan.
  • Amari S; Department of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan.
  • Maruyama H; Department of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan.
  • Tsukamoto K; Department of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan.
  • Isayama T; Department of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan.
  • Sakamoto K; Chilldren's Cancer Center, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan.
  • Shioda Y; Chilldren's Cancer Center, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan.
  • Miyazaki O; Department of Radiology, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan.
  • Irie R; Department of Clinical Pathology, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan.
  • Yoshioka T; Department of Pathology, Nippon Koukan Hospital, 1-2-1 Koukandouri, Kawasaki-ku, Kawasaki City, Kanagawa, 210-0852, Japan.
  • Mochimaru N; Department of Clinical Pathology, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan.
  • Yoshida K; Department of Dermatology, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan.
  • Ito Y; Department of Dermatology, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan.
BMC Pediatr ; 21(1): 161, 2021 04 06.
Article en En | MEDLINE | ID: mdl-33823829
BACKGROUND: Systemic juvenile xanthogranuloma is a very rare disease typically presents as skin lesions with yellow papules or nodules and is sometimes fatal. We report a case of congenital neonatal systemic juvenile xanthogranuloma with atypical skin appearance that made the diagnosis difficult. CASE PRESENTATION: A preterm Japanese female neonate with prenatally diagnosed fetal hydrops in-utero was born with purpuric lesions involving the trunk and face. Since birth, she had hypoxemic respiratory failure, splenomegaly, anemia, thrombocytopenia, coagulopathy, and was transfusion dependent for red blood cells, fresh frozen plasma, and platelets. Multiple cystic lesions in her liver, part of them with vascular, were detected by ultrasound. A liver biopsy was inconclusive. A skin lesion on her face similar to purpura gradually changed to a firm and solid enlarged non-yellow nodule. Technically, the typical finding on skin biopsy would have been histiocytic infiltration (without Touton Giant cells) and immunohistochemistry results which then would be consistent with a diagnosis of systemic juvenile xanthogranuloma, and chemotherapy improved her general condition. CONCLUSIONS: This case report shows that skin biopsies are necessary to detect neonatal systemic juvenile xanthogranuloma when there are organ symptoms and skin eruption, even if the skin lesion does not have a typical appearance of yellow papules or nodules.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Púrpura / Xantogranuloma Juvenil Tipo de estudio: Diagnostic_studies Límite: Female / Humans / Newborn Idioma: En Revista: BMC Pediatr Asunto de la revista: PEDIATRIA Año: 2021 Tipo del documento: Article País de afiliación: Japón Pais de publicación: Reino Unido
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Púrpura / Xantogranuloma Juvenil Tipo de estudio: Diagnostic_studies Límite: Female / Humans / Newborn Idioma: En Revista: BMC Pediatr Asunto de la revista: PEDIATRIA Año: 2021 Tipo del documento: Article País de afiliación: Japón Pais de publicación: Reino Unido