Multiorgan Langerhans cell histiocytosis (LCH) presenting in the neonatal period is associated with a poor prognosis. We report a 5-day-old neonate who presented at birth with diffuse cutaneous nodules and respiratory failure who received a postmortem diagnosis of LCH. We emphasize the importance of recognizing the cutaneous findings of this rare disease.
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Histiocytosis are caused by pathogenic myeloid cells, and can be classified as Langerhans cell histiocytosis (LCH) and non-LCH. Erdheim-Chester disease (ECD) is a non-LCH, characterized by multi-organ involvement, typical imaging findings, and confirmatory histological studies. A case with multi-organ involvement and histological confirmation is presented.
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