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Congenital systemic Langerhans cell histiocytosis presenting as hydrops fetalis
Author(s) -
Lee Cheuk H,
Lau Tze K,
To Ka F,
Lam Hugh S,
Chan Anthony WH,
Ng Pak C
Publication year - 2005
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.2005.tb01866.x
Subject(s) - hydrops fetalis , medicine , fulminant , langerhans cell histiocytosis , pediatrics , histiocytosis , rare disease , disease , fetus , pathology , pregnancy , surgery , genetics , biology
Congenital Langerhans cell histiocytosis (LCH) encompasses a wide spectrum of disease involvement and severity. Congenital “self‐healing” cutaneous LCH represents one end of the spectrum, whereas the case we encountered represents the other extreme. A rare case of congenital LCH with severe multiorgan involvement presenting as hydrops fetalis is described in this report. Hydrops fetalis has not previously been associated with congenital LCH. The overall clinical features of this infant closely mimicked those of disseminated congenital infection, and he ran a fulminant and rapidly fatal course. Conclusion: A high index of suspicion is required to diagnose congenital LCH in the early neonatal period. Hydrops fetalis is an ominous sign and probably reflects severe systemic disease compromising the well‐being of the fetus.