Clinical Manifestations of Letterer‐siwe Disease in the Neonatal Period

We'itrell, G.; Svenningsen, N. W.; Nordenfelt, Erik; Lindholm, Karin

doi:10.1111/j.1651-2227.1973.tb08112.x

Cited by 7 publications

(1 citation statement)

References 8 publications

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“…He subsequently ran a rapidly fatal downhill course consistent with patients suffering from multisystemic LCH. As in previous fatal cases 1, 6–9, prematurity, early age of symptom onset and multiorgan involvement, in particular severe pulmonary disease, predict a poor prognosis. To our knowledge, this is the first report of LCH presenting as hydrops fetalis detected by antenatal ultrasound.…”

Section: Discussionsupporting

confidence: 52%

Congenital systemic Langerhans cell histiocytosis presenting as hydrops fetalis

Lee

Lau

et al. 2005

Acta Paediatrica

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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.

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Section: Discussionsupporting

confidence: 52%

Congenital systemic Langerhans cell histiocytosis presenting as hydrops fetalis

Lee

Lau

et al. 2005

Acta Paediatrica

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Congenital Self-healing Histiocytosis

Bonifazi¹

1982

Arch Dermatol

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Congenital Letterer-Siwe disease associated with protein losing enteropathy

et al. 1980

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A case of congenital Letterer-Siwe disease associated with protein losing enteropathy is presented. The patient was born at term after an uneventful pregnancy, and generalized eczematous skin eruptions were observed at birth. Excessive protein loss from the intestine was recognized. A cutaneous biopsy from typical lesions revealed histiocytic infiltrations and Langerhans' cell granules in the cytoplasm of the histiocytes.

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Clinical Manifestations of Letterer‐siwe Disease in the Neonatal Period

Cited by 7 publications

References 8 publications

Congenital systemic Langerhans cell histiocytosis presenting as hydrops fetalis

Congenital systemic Langerhans cell histiocytosis presenting as hydrops fetalis

Congenital Self-healing Histiocytosis

Congenital Letterer-Siwe disease associated with protein losing enteropathy

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