Langerhans cell histiocytosis in a premature baby presenting with skin‐isolated disease: case report and literature review

Abstract

Careful observation should be applied to newborns with skin-only Langerhans cell histiocytosis in order to identify in time progression to potentially fatal systemic disease.

Two case report studies of Langerhans cell histiocytosis with an analysis of 918 patients of Langerhans cell histiocytosis in literatures published in China†

“…In the 918 cases, other systemic manifestations were found, of which fever, hepatomegaly, splenomegaly, bone damage, and pulmonary involvement were the most common. Rash varies in its manifestations, and a differential diagnosis with the following should be noted: infantile eczema, seborrheic dermatitis, infectious mononucleosis, juvenile rheumatoid arthritis (Still’s disease), malignant histiocytosis, sepsis, and sarcoidosis 6,7,8 . Skin biopsy is the most frequently used method to make a definite diagnosis of rash.…”

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 tissue examined histologically and may provide the 1st diagnostic evidence of disease.…”

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

“…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).…”

“…These poor outcomes were mostly associated with pulmonary failure or multiorgan failure, with two cases of hydrops fetalis. 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].…”

“…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].…”