A Fatal Case of Congenital Langerhans Cell Histiocytosis with Disseminated Cutaneous Lesions in a Premature Neonate.

Michio Inoue,Shinsaku Imashuku,Tsuyoshi Egawa,Tomoaki Ioroi,Masaaki Kugo,Yoko Tomita

doi:10.1155/2016/4972180

Abstract

Background. The outcome of neonates with congenital cutaneous Langerhans cell histiocytosis (LCH) is variable. Observations. We report a case of LCH in a female premature neonate born at 33-week gestation. She had disseminated cutaneous lesions, which consisted of hemorrhagic papules and vesicles, with sparse healthy skin areas, and the hands and feet were contracted with scarring and blackened. She was in respiratory failure although no apparent pulmonary or bone lesions on X-rays were noted. Skin biopsy confirmed a diagnosis of LCH due to observation of CD1a+ Langerhans cells, which lacked expression of E-cadherin and CD56. The patient died 57 hours after birth. Conclusions. Based on this case and the literature survey, the outcome of premature babies with congenital cutaneous LCH lesions is noted to be unfavorable, with the majority of such cases suffering from multisystem disease.

Highlights

Langerhans cell histiocytosis (LCH) is characterized by lesions that include CD1a+CD207+ dendritic cells, along with inflammatory cell infiltrates
We report a fatal case of congenital LCH in a prematurely born female neonate with disseminated cutaneous lesions resembling severe burns, but with no apparent pulmonary or bone lesions
LCH should be considered in the differential diagnosis of widespread cutaneous lesions in neonates

Summary

Background

The outcome of neonates with congenital cutaneous Langerhans cell histiocytosis (LCH) is variable. We report a case of LCH in a female premature neonate born at 33-week gestation. She had disseminated cutaneous lesions, which consisted of hemorrhagic papules and vesicles, with sparse healthy skin areas, and the hands and feet were contracted with scarring and blackened. She was in respiratory failure no apparent pulmonary or bone lesions on X-rays were noted. Based on this case and the literature survey, the outcome of premature babies with congenital cutaneous LCH lesions is noted to be unfavorable, with the majority of such cases suffering from multisystem disease

Introduction

Case Report

Discussion