[Langerhans cell histiocytosis: Letterer-Siwe disease - the importance of dermatological diagnosis in two cases]. / Histiocitose de células de Langerhans: doença de Letterer-Siwe - importância do diagnóstico dermatológico em dois casos.

Langerhans cell histiocytosis is defined as a clonal proliferation of Langerhans phenotypic-like cells. Letterer-Siwe disease is the most common and serious of these entities, affecting mainly infants up to two years of age. We present two cases of this rare disease, diagnosed after dermatological examination, highlighting its typical aspects.

Histiocitose de células de Langerhans: doença de Letterer-Siwe - importância do diagnóstico dermatológico em dois casos / Langerhans cell histiocytosis: Letterer-Siwe disease - the importance of dermatological diagnosis in two cases

A histiocitose de células de Langerhans é proliferação clonal de células fenotipicamente semelhantes às células de Langerhans. Anteriormente denominada Letterer-Siwe, é a forma mais comum e mais grave dessa enfermidade, acometendo sobretudo crianças até os dois anos de idade. São apresentados dois casos dessa rara doença, diagnosticados após parecer dermatológico, destacando-se seus aspectos mais característicos.

Langerhans cell histiocytosis is defined as a clonal proliferation of Langerhans phenotypic-like cells. Letterer-Siwe disease is the most common and serious of these entities, affecting mainly infants up to two years of age. We present two cases of this rare disease, diagnosed after dermatological examination, highligthing its typical aspects.