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Indeterminate Cell Histiocytosis: A Rare Histiocytic Disorder

1997; Lippincott Williams & Wilkins; Volume: 19; Issue: 3 Linguagem: Inglês

10.1097/00000372-199706000-00014

1533-0311

Liborio Manente, Claudia Cotellessa, Iris Schmitt, Ketty Peris, G Torlone, Andrea Onetti Muda, Maria Concetta Romano, Sergio Chementi,

Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis

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.