Raper R F, Ibels L S
Nephron. 1985;39(4):389-92. doi: 10.1159/000183411.
Widespread, progressive skin necrosis developed in a 42-year-old male with a 5-year history of osteosclerotic myeloma. Biopsy of the necrotic lesions demonstrated a leucocytoclastic vasculitis with extensive vascular calcification. Radiological investigations demonstrated widespread arterial calcification. Clinical improvement of the established skin lesions followed the institution of a forced calciuresis and parathyroid hormone suppression by induced hypermagnesaemia and phosphate depletion. No further cutaneous necrosis developed. Subsequent treatment with oral immunosuppressive therapy and the diphosphonate, EHDP, has been associated with a complete 18-month remission. The relationship of this apparently unique pathological process to the osteosclerotic myeloma is discussed, together with the rationale for the therapeutic regime instituted.
一名患有骨硬化性骨髓瘤5年的42岁男性出现了广泛的、进行性皮肤坏死。对坏死病变进行活检显示为白细胞破碎性血管炎,并伴有广泛的血管钙化。影像学检查显示广泛的动脉钙化。通过诱导高镁血症和磷酸盐耗竭来促进钙排泄和抑制甲状旁腺激素后,已形成的皮肤病变出现临床改善。未再发生皮肤坏死。随后口服免疫抑制治疗和二膦酸盐EHDP治疗使病情完全缓解达18个月。本文讨论了这一明显独特的病理过程与骨硬化性骨髓瘤的关系,以及所采用治疗方案的理论依据。