Zouboulis C C, Blume-Peytavi U, Lennert T, Stavropoulos P G, Schwarz A, Runkel N, Trautmann C, Orfanos C E
Department of Dermatology, University Medical Center Benjamin Franklin, Free University of Berlin, Germany.
Br J Dermatol. 1996 Oct;135(4):617-22.
Metastatic calcinosis is a common feature of chronic renal failure. Its first manifestations are bone demineralization and non-visceral and/or visceral calcification with mostly mural deposits in arteries and arterioles. It is initially characterized by hyperphosphataemia followed by secondary or tertiary hyperparathyroidism. Cutaneous involvement is a rare complication. Histologically, the lesions show vascular calcification with ischaemic skin necrosis. Extreme cases may produce calcinosis cutis (calciphylaxis), i.e. disseminated calcification of the subcutaneous tissue and dermis in the form of hard painful cutaneous nodules and plaques with subsequent ulceration. Metastatic calcinosis is a disease affecting adults, while the dystrophic or idiopathic type can develop in children. We present the case of a 6-year-old boy with end-stage renal disease, attributed to congenital renal hypoplasia, and accompanied by secondary hyperparathyroidism. He developed fulminant tertiary hyperparathyroidism and metastatic calcinosis of the lungs, as well as cutaneous necrosis of the buttocks and legs, subsequent to calcification of arteries and arterioles. A maternal renal transplant failed to function. The serum parathormone, calcium and phosphate levels could not be controlled by maintenance dialysis, phosphate binders and calcitriol. Total parathyroidectomy without autotransplantation of parathyroid tissue rapidly returned the serum parathormone, calcium and phosphate levels to normal. In addition, topical treatment using merbromine solution and hydrocolloid dressings, healed the ulcers with significant scar formation, within 2.5 months after parathyroidectomy. A renewed increase of the calcium x phosphate product, 2 months after parathyroidectomy, was attributed to mobilization of calcium compounds from the viscera, as confirmed by a chest X-ray.
转移性钙化是慢性肾衰竭的常见特征。其最初表现为骨质脱矿以及非内脏和/或内脏钙化，主要为动脉和小动脉的壁内沉积。其最初特征为高磷血症，随后继发或转为甲状旁腺功能亢进。皮肤受累是一种罕见的并发症。组织学上，病变表现为血管钙化伴缺血性皮肤坏死。极端情况下可产生皮肤钙化（calciphylaxis），即皮下组织和真皮呈弥漫性钙化，表现为坚硬疼痛的皮肤结节和斑块，随后发生溃疡。转移性钙化是一种成人疾病，而营养不良性或特发性类型可在儿童中发生。我们报告一例6岁男孩，患有终末期肾病，病因是先天性肾发育不全，并伴有继发性甲状旁腺功能亢进。他在动脉和小动脉钙化后，发展为暴发性三发性甲状旁腺功能亢进和肺部转移性钙化，以及臀部和腿部皮肤坏死。母亲的肾移植未能发挥功能。维持性透析、磷结合剂和骨化三醇无法控制血清甲状旁腺激素、钙和磷水平。未进行甲状旁腺组织自体移植的甲状旁腺全切术迅速使血清甲状旁腺激素、钙和磷水平恢复正常。此外，在甲状旁腺切除术后2.5个月内，使用汞溴红溶液和水胶体敷料进行局部治疗，使溃疡愈合并形成明显瘢痕。甲状旁腺切除术后2个月，钙磷乘积再次升高，经胸部X线证实，这归因于内脏中钙化合物的动员。
