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Congenital Self-Healing Langerhans Cell Histiocytosis: A Rare Presentation of Blueberry Muffin Baby “Spectrum”

A case of congenital self-healing Langerhans cell histiocytosis (CSHLCH), also known as Hashimoto-Pritzker disease, is reported. The newborn presented as blueberry muffin baby at birth, showing numerous non-blanching blue-purplish and dark-red papular, nodular lesions without documented infections a...

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Autores principales: Hansel, Katharina, Tramontana, Marta, Troiani, Stefania, de Benedictis, Diletta, Bianchi, Leonardo, Cucchia, Rosa, Simonetti, Stefano, Stingeni, Luca
Formato: Online Artículo Texto
Lenguaje:English
Publicado: S. Karger AG 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6547258/
https://www.ncbi.nlm.nih.gov/pubmed/31192198
http://dx.doi.org/10.1159/000499311
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author Hansel, Katharina
Tramontana, Marta
Troiani, Stefania
de Benedictis, Diletta
Bianchi, Leonardo
Cucchia, Rosa
Simonetti, Stefano
Stingeni, Luca
author_facet Hansel, Katharina
Tramontana, Marta
Troiani, Stefania
de Benedictis, Diletta
Bianchi, Leonardo
Cucchia, Rosa
Simonetti, Stefano
Stingeni, Luca
author_sort Hansel, Katharina
collection PubMed
description A case of congenital self-healing Langerhans cell histiocytosis (CSHLCH), also known as Hashimoto-Pritzker disease, is reported. The newborn presented as blueberry muffin baby at birth, showing numerous non-blanching blue-purplish and dark-red papular, nodular lesions without documented infections and systemic involvement. Histopathological and immunohistochemical findings were suggestive for Langerhans cell histiocytosis. During the first 12 weeks of life, the cutaneous lesions progressively and spontaneously regressed with some atrophic scars. One-year follow-up is negative for relapse of cutaneous lesions or systemic involvement, confirming the diagnosis of CSHLCH.
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spelling pubmed-65472582019-06-12 Congenital Self-Healing Langerhans Cell Histiocytosis: A Rare Presentation of Blueberry Muffin Baby “Spectrum” Hansel, Katharina Tramontana, Marta Troiani, Stefania de Benedictis, Diletta Bianchi, Leonardo Cucchia, Rosa Simonetti, Stefano Stingeni, Luca Dermatopathology (Basel) Case Report A case of congenital self-healing Langerhans cell histiocytosis (CSHLCH), also known as Hashimoto-Pritzker disease, is reported. The newborn presented as blueberry muffin baby at birth, showing numerous non-blanching blue-purplish and dark-red papular, nodular lesions without documented infections and systemic involvement. Histopathological and immunohistochemical findings were suggestive for Langerhans cell histiocytosis. During the first 12 weeks of life, the cutaneous lesions progressively and spontaneously regressed with some atrophic scars. One-year follow-up is negative for relapse of cutaneous lesions or systemic involvement, confirming the diagnosis of CSHLCH. S. Karger AG 2019-05-07 /pmc/articles/PMC6547258/ /pubmed/31192198 http://dx.doi.org/10.1159/000499311 Text en Copyright © 2019 by S. Karger AG, Basel http://creativecommons.org/licenses/by-nc/4.0/ This article is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License (CC BY-NC-ND) (http://www.karger.com/Services/OpenAccessLicense). Usage and distribution for commercial purposes requires written permission.
spellingShingle Case Report
Hansel, Katharina
Tramontana, Marta
Troiani, Stefania
de Benedictis, Diletta
Bianchi, Leonardo
Cucchia, Rosa
Simonetti, Stefano
Stingeni, Luca
Congenital Self-Healing Langerhans Cell Histiocytosis: A Rare Presentation of Blueberry Muffin Baby “Spectrum”
title Congenital Self-Healing Langerhans Cell Histiocytosis: A Rare Presentation of Blueberry Muffin Baby “Spectrum”
title_full Congenital Self-Healing Langerhans Cell Histiocytosis: A Rare Presentation of Blueberry Muffin Baby “Spectrum”
title_fullStr Congenital Self-Healing Langerhans Cell Histiocytosis: A Rare Presentation of Blueberry Muffin Baby “Spectrum”
title_full_unstemmed Congenital Self-Healing Langerhans Cell Histiocytosis: A Rare Presentation of Blueberry Muffin Baby “Spectrum”
title_short Congenital Self-Healing Langerhans Cell Histiocytosis: A Rare Presentation of Blueberry Muffin Baby “Spectrum”
title_sort congenital self-healing langerhans cell histiocytosis: a rare presentation of blueberry muffin baby “spectrum”
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6547258/
https://www.ncbi.nlm.nih.gov/pubmed/31192198
http://dx.doi.org/10.1159/000499311
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