A 64-year-old woman, otherwise healthy, presented with multiple reddis
h-brown, slightly yellowish papules on the face and neck, which had de
veloped 3 years earlier. The lesions were painless and nonpruritic and
varied in diameter from 1 to 5 mm. Histological and immunohistochemic
al examination of cutaneous biopsies revealed a diffuse dermal infiltr
ate composed mainly of histiocytes which expressed both Langerhans cel
l as well as monocytic/macrophages cell marker characteristics. Electr
on microscopic studies revealed no Birbeck granules within the cylopla
sm of the neoplastic cells. leading to a diagnosis of indeterminate ce
ll histiocytosis. Indeterminate cell histiocytosis is a very rare dise
ase characterized by the proliferation of indeterminate histiocytes wh
ich morphologically and immunophenotypically resemble Langerhans cells
but lack Birbeck granules.