Vol. 9, Issue 1 (2020)

Author(s):
Vindhyvasni, Virginia Paul and Alka Gupta

Abstract:
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. The most frequent LCH occurs in children between 1 and 3 years of age, although it may occur at any age. Its annual incidence seems to be between 2 to 5 cases per million children. It is twice more frequent among males. Its pathogenesis remains unknown. Its reactional or neoplastic nature is still debated, although there are more arguments favoring the first option.