
Congenital Self-Healing Langerhans Cell Histiocytosis: A Rare Presentation of Blueberry Muffin Baby “Spectrum”
Author(s) -
Hansel Katharina,
Tramontana Marta,
Troiani Stefania,
de Benedictis Diletta,
Bianchi Leonardo,
Cucchia Rosa,
Simonetti Stefano,
Stingeni Luca
Publication year - 2019
Publication title -
dermatopathology
Language(s) - English
Resource type - Journals
ISSN - 2296-3529
DOI - 10.1159/000499311
Subject(s) - 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.