Congenital systemic Langerhans cell histiocytosis presenting as hydrops fetalis

Abstract

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.

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

“…Skin lesions were variable, from diffuse cutaneous nodules [11], isolated vesiculopapulomacular rash [12], generalized vesicles [9], papular rash [13], and disseminated burn-like lesions (present case). Of the 2 cases associated with hydrops fetalis, one died early (36 hours), while the other in 12 days [8, 9]. Exceptionally, a premature baby with cutaneous lesions at birth (albeit less extensive than in our case) survived the neonatal period [13].…”

“…Although cutaneous lesions associated with LCH in infants are often self-healing [5], evidence suggests that the occurrence of these lesions in association with premature birth is not benign [8–12] (Table 1). As summarized, in this case and in six other cases identified in the literature, the majority of premature neonates with congenital cutaneous LCH lesions had a multisystem disease and poor outcome (Table 1).…”

“…Congenital LCH that is limited to cutaneous lesions is generally thought to be clinically benign, with a good prognosis [5], but rare cases have a poor outcome [6, 7]. Notably, congenital cutaneous LCH in preterm babies (born before 37-week gestation) is a severe, systemic disease that usually causes death in utero or after delivery [8–12]. In some premature neonates with LCH, lethal hydrops fetalis may develop [8, 9].…”

“…Notably, congenital cutaneous LCH in preterm babies (born before 37-week gestation) is a severe, systemic disease that usually causes death in utero or after delivery [8–12]. In some premature neonates with LCH, lethal hydrops fetalis may develop [8, 9]. Here, 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.…”

Is Langerhan Cell Histiocytosis Complicated with Hydrops Fetalis Exclusively Lethal in Premature Neonates?

“…Patients <1 year of age with multiple organ involvement are at high risk of death (2). Preterm infants with LCH often possess rapidly deteriorated course with extensive pulmonary involvement (3). We report a mortality case of a premature baby who suffered from congenital Langerhans cell histiocytosis, with compromised lung condition presenting as hydrops fetalis with pleural effusion and persistent pulmonary hypertension (PPHN).…”