Indeterminate cell histiocytosis: a rare histiocytic disorder.
A 64-year-old woman, otherwise healthy, presented with multiple reddish-brown, slightly yellowish papules on the face and neck, which had developed 3 years earlier. The lesions were painless and nonpruritic and varied in diameter from 1 to 5 mm. Histological and immunohistochemical examination of cutaneous biopsies revealed a diffuse dermal infiltrate composed mainly of histiocytes which expressed both Langerhans cell as well as monocytic/macrophages cell marker characteristics. Electron microscopic studies revealed no Birbeck granules within the cytoplasm of the neoplastic cells, leading to a diagnosis of indeterminate cell histiocytosis. Indeterminate cell histiocytosis is a very rare disease characterized by the proliferation of indeterminate histiocytes which morphologically and immunophenotypically resemble Langerhans cells but lack Birbeck granules.
Manente
L
L
Department of Dermatology, University of L'Aquila, Italy.
Cotellessa
C
C
Schmitt
I
I
Peris
K
K
Torlone
G
G
Muda
A O
AO
Romano
M C
MC
Chementi
S
S
D005260:Female; D015614:Histiocytosis / Q000473:pathology*; D006801:Humans; D008875:Middle Aged; D012867:Skin / Q000648:ultrastructure