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

Authors :
Katharina Hansel
Marta Tramontana
Stefania Troiani
Diletta de Benedictis
Leonardo Bianchi
Rosa Cucchia
Stefano Simonetti
Luca Stingeni
Source :
Dermatopathology, Vol 6, Iss 2, Pp 37-40 (2019)
Publication Year :
2019
Publisher :
MDPI AG, 2019.

Abstract

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.

Details

Language :
English
ISSN :
22963529
Volume :
6
Issue :
2
Database :
Directory of Open Access Journals
Journal :
Dermatopathology
Publication Type :
Academic Journal
Accession number :
edsdoj.9585d09bebbe4f59b77e3d92a3434b92
Document Type :
article
Full Text :
https://doi.org/10.1159/000499311