Abstract
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 autosomal dominant, and by other clinical and histologic features. Since a storage disease could be excluded, the syndrome presented here seems to be a hitherto unknown entity among the benign generalized histiocytoses.
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