A fatal case of congenital disseminated langerhans cell histiocytosis

Abstract

The case of a newborn male infant with congenital Langerhans cell histiocytosis (LCH) is presented. At birth, showed cutaneous lesions (papules and vesicles with a haemorrhagic aspect), mucosal and ganglionic involvement. Biopsy of these lesions led to the diagnosis of LCH. At 24 hours of life the patient began with respiratory, hepatic, hematological and renal dysfunction, and died at 72 hours of life, despite corticoid treatment. LHC with vesicles and a rapid and fatal development, has previously only been described in three patients. The differential diagnosis of a disseminated and hemorrhagic vesicular eruption in a newborn infant is extensive and must include LHC.

Congenital systemic Langerhans cell histiocytosis presenting as hydrops fetalis

“…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.…”

Congenital Langerhans Cell Histiocytosis with Placental Involvement

“…Multisystem LCH typically has a poor prognosis with a fulminant disease course. Congenital presentations of LCH are exceedingly rare and can mimic more common entities, such as sepsis, neuroblastoma, and leukemia cutis, which may delay both recognition and appropriate treatment [3][4][5][6][7][8]. In cases of congenital LCH, the placenta is likely to be the 1st fetal * Corresponding author, e-mail: jterry@hhsc.ca tissue examined histologically and may provide the 1st diagnostic evidence of disease.…”

Congenital systemic Langerhans cell histiocytosis presenting as hydrops fetalis

“…He subsequently ran a rapidly fatal downhill course consistent with patients suffering from multisystemic LCH. As in previous fatal cases [1,[6][7][8][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.…”

“…By definition, these patients should have a good prognosis. However, LCH represents a much wider spectrum of disease manifestations, ranging from benign to fulminant, localized to extracutaneous multiorgan involvement, and self-healing to rapidly fatal [1][2][3][4][5][6][7][8]. Further, multisystemic and lethal disease can initially be indistinguishable from isolated cutaneous LCH [1].…”