Histiocitosis de células de Langerhans, con compromiso de un único órgano

Resumen La histiocitosis de celulas de Langerhans con compromiso de la piel como unico organo, tambien llamada reticulohistiocitosis congenita autoinvolutiva o enfermedad de Hashimoto-Pritzker, es una variante de las histiocitosis de celulas de Langerhans con compromiso cutaneo primario. Se presenta el caso de un neonato con lesiones papulo-nodulares eritematosas, localizadas en cabeza, tronco y miembros. La biopsia de piel mostro un infi ltrado con celulas de Langerhans. Los examenes complementarios descartaron compromiso extracutaneo. Enfatizamos la importancia de realizar un seguimiento a largo plazo de esta entidad por la posibilidad de progresion o recaida cutanea o extracutaneas (Dermatol Argent 2010;16 Supl 2:44-46). Palabras clave: histiocitosis de celulas de Langerhans cutanea con compromiso de un unico organo, reticulohistiocitosis congenita autoinvolutiva, enfermedad de Hashimoto Pritzker Title: Cutaneous Langerhans’-cell histiocytosis with single-organ–system involvement Abstract Cutaneous Langerhans’-cell histyocitosis with single-organ–system involvement, congenital self-healing reticulohistiocytosis or Hashimoto-Pritzker disease is a variant of Langerhans’-cell histyocitosis with primarycutaneous involvement. We report a case of a newborn male with erithematous papules and nodules located on the head, trunk and limbs. The skin biopsy showed an infiltrate with Langerhans cells. Extracutaneous involment was absent. We emphasize the importance of long-term follow-up, because of the possibility of eventual progression or relapse, either in the skin or at extracutaneous sites (Dermatol Argent 2010;16 Supl 2:44-46). Key words: cutaneous Langerhans’-cell histyocitosis with single-organ–system involvement, congenital self-healing reticulohistiocytosis, Hashimoto-Pritzker disease.