0000000000300794
AUTHOR
Nikolaus Hoede
Hereditary Progressive Mucinous Histiocytosis in Women
We describe three female patients in a family of two generations, who suffered from generalized and maximally pea-sized histiocytic tumors beginning in early adolescence. The disease ran a uniform and slowly progressive course and was confined to the skin. There were no signs of spontaneous tumor regression. Histologic, immunohistochemical, and ultrastructural examination revealed the histiocytic nature of the tumors. An outstanding finding was a marked production of mucinous material, predominantly in long-standing tumors. This nonlangerhansian syndrome differs from other benign normolipemic histiocytic diseases with generalized histiocytic tumors by inheritance, which is most likely autos…
Epithelial markers and differentiation in adnexal neoplasms of the skin: an immunohistochemical study including individual cytokeratins
Applying immunohistochemical procedures for the detection of eight different cytokeratin (CK) polypeptides and other differentiation markers, we compared the staining patterns of normal cutaneous structures with those of benign adnexal tumors (n = 65). Syringomas exhibited a marker pattern highly reminiscent of that seen in normal dermal eccrine ducts (EMA in peripheral cells, CK 10 in intermediate cells, and CK 6, CK 19, and CEA in luminal cells). Nodular hidradenomas exhibited complex patterns suggesting relationship between tumor cells, including clear cells, and normal secretory coil cells (CK 7, CK 8, CK 19, and EMA); however, dermal-duct and epidermoid differentiation were also detect…