Bork and Hoede (1988) described 3 patients, 2 sisters and the daughter of 1, who suffered from generalized and maximally pea-sized histiocytic tumors beginning in early adolescence. The disorder ran a uniform, slowly progressive course and was confined ... Bork and Hoede (1988) described 3 patients, 2 sisters and the daughter of 1, who suffered from generalized and maximally pea-sized histiocytic tumors beginning in early adolescence. The disorder ran a uniform, slowly progressive course and was confined to the skin. There were no signs of spontaneous tumor regression, and the tumors showed a marked reduction of mucinous material. In another family, Bork (1994) described the disorder in a 52-year-old woman and her 25-year-old daughter. Both showed longstanding lesions without tumor regression. There was no evidence of visceral involvement. Histologic, immunohistochemical, and ultrastructural examinations revealed histiocytes from the monocyte/macrophage subset as the main constituents of the tumors. The cells contained abundant zebra and myeloid bodies and vacuoles, indicating a lysosomal storage disease. Biochemical and enzymatic tests showed no other evidence of a mucopolysaccharidosis.