Congenital Langerhans cell histiocytosis: a good prognosis disease?

Abstract

Langerhans cell histiocytosis is rare and more frequent in children. The skin is affected in 50% of the cases and is the only site in 10%. Its course varies from self-limited and localized forms to severe multisystemic forms. Congenital cases are usually exclusively cutaneous and self-limited, with spontaneous remission in months. This study presents a rare congenital case, initially restricted to the skin, with subsequent dissemination and fatal outcome. A male newborn presented congenital disseminated erythematous scaly lesions. The biopsy was conclusive for Langerhans cell histiocytosis. The patient evolved into the multisystemic form in weeks, when chemotherapy was started, according to the LCH-2009 protocol; however, the patient was refractory to treatment and died.

Histiocitosis congénita de células de Langerhans

“…La histiocitosis de células de Langerhans es un trastorno raro cuya incidencia real se desconoce 7 , aunque se reporta una de 3 a 5 casos por millón, especialmente en la infancia. En el periodo neonatal, esta es, aproximadamente, de 1 a 2 casos por millón 10 y es ligeramente superior en hombres 4 . La incidencia real en Colombia no se conoce.…”

“…El diagnóstico se confirma mediante los estudios histopatológicos y el análisis por inmunohistoquímica con resultado positivo para las moléculas CD1 a CD207 (langerina) características de las líneas dendríticas, así como mediante otros marcadores de la línea mieloide, como las proteínas S100 y CD68 9 , 10 , 15 . La historia clínica, el examen físico y las pruebas de laboratorio deben correlacionarse para clasificar al paciente según el grado de afectación y, así, proceder con el tratamiento 5 .…”

BRAF V600E-Positive Congenital Multisite Langerhans Cell Histiocytosis

“…Langerhans cell histiocytosis (LCH) is a rare entity resulting from the clonal proliferation of cells similar to Langerhans cells (LC) [1,2]. Its incidence is 3 to 5 cases per million [2].…”

“…Its incidence is 3 to 5 cases per million [2]. It can appear at any age, but it is more frequent in childhood [1,2]. In the neonatal period, the incidence is one to two cases per million [1,2].…”

“…It can appear at any age, but it is more frequent in childhood [1,2]. In the neonatal period, the incidence is one to two cases per million [1,2]. Depending on clinical presentations, it is further distinguished into including Letterer-Siwe disease, Hand-Schüller Christian disease, eosinophilic granuloma, and a congenital self-healing form [3].…”