A UNIQUE ERYTHERMALGIA IN A PATIENT WITH VON RECKLINGHAUSEN NEUROFIBROMATOSIS

We report a 58-year-old female patient with von Recklinghausen neurofibromatosis who developed a condition similar to that of erythermalgia in many respects, especially on her hands. The distress worsened upon exposure to cold and improved when warmed. She felt pain over the hands restricted to the time of urination when her bladder was full. It was found that her distress was felt when blood was unstably forced through partial closing of abnormal a-v shunts into diseased blood vessels of the distal extremities and that the a-v shunts of this patient apparently worked differently.