A Case Indistinguishable Between Indeterminate Cell Histiocytosis and Cutaneous Rosai-Dorfman Disease

作者：Kuzyk Alexandra; Silver Shane; Wallace Sharon; Hamza Sate; Toole John^*

来源：Journal of Cutaneous Medicine and Surgery, 2017, 21(1): 68-71.

DOI：10.1177/1203475416664038

• histiocytosis
• indeterminate cell histiocytosis
• cutaneous Rosai-Dorfman
• sinus histiocytosis with massive lymphadenopathy
• non-Langerhans cell histiocytosis

Histiocytic disorders are characterized by the accumulation of macrophages or dendritic cells, 2 types of antigen-presenting cells derived from CD34+ progenitors, in various tissues.(1,2) There are over 100 described subtypes of histiocytosis with a wide range and significant overlap of clinical and histopathologic features. (1,3) Langerhans cells are epithelial dendritic cells(4) characterized by strong S-100 protein and CD1a immuno-staining.(5) Langerhans cells are the predominant component of Langerhans cell histiocytosis (LCH). Macrophages usually lack the Langerhans cell markers but express CD68 and Ki-M1p.(6) Histiocytic disorders with a predominant macrophage component are called non-Langerhans cell histiocytosis (NLCH).
Indeterminate cell histiocytosis (ICH) is a rare proliferative disorder of histiocytes that usually presents as a cutaneous disease with multiple pink to brown papules and nodules. 7 ICH is characterized by immunophenotypic features of both Langerhans cells and macrophages and illustrates overlap between LCH and NLCH.(2,8) Cutaneous Rosai-Dorfman disease (CRDD) is a very rare variant of sinus histiocytosis with massive lymphadenopathy (SHML).(9) SHML is a type of NLCH. CRDD is restricted to the skin and can present as single or multiple red-brown papules and nodules.(10) The lesional histiocytes typically stain S-100 protein positive, CD68 positive, and CD1a negative.(11)

• 出版日期2017-2