[Langerhans' cell histiocytosis: various manifestations with the same histopathologic base].
Langerhans' cell histiocytosis (LCH), previously known as histiocytosis X, is a rare disease. It is characterized by the accumulation and proliferation of histiocytes, eosinophils and Langerhans' cells with Birbeck granules detected by electron microscopy. It involves single organs or systems or can present as a multisystem disease. The clinical presentation may vary widely, ranging from benign self-limiting types with spontaneous regression to slowly-progressive malignant disease. We report five cases of LCH with the same histopathologic basis but different outcome.
Martínez Baylach
J
J
Servicio de Pediatría. Universitat Autònoma de Barcelona. Hospital de la Santa Creu i Sant Pau. Barcelona. España. jmar9072@hotmail.com
Pardo García
N
N
Torrent Español
M
M
Moliner Calderón
E
E
Anquela Sanz
I
I
Cubells Rieró
J
J
D002648:Child; D002675:Child, Preschool; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D016896:Treatment Outcome