Gebska Edyta, Vanaga-Besser Santa, Zajeck Wojciech, Matuszewska Gabriela, Jarzab Jerzy
Silesian Medical University in Katowice, Department of Internal Medicine, Dermatology and Allergology, Zabrze, Poland.
Pol Merkur Lekarski. 2009 Aug;27(158):123-8.
Calcyphylaxis consists in vascular calcium deposition and skin necrosis, which appears in the final stage of renal failure. Advanced forms of systemic connective tissue disease such as lupus erythematosus, especially with concomitant vasculitis, leading to renal failure and uremia may be conducive to calcyphylaxis. We are presenting a case of a 50-year-old male requiring chronic peritoneal dialysis since the age of 43, due to end-stage renal disease resulting from chronic glomeluronephritis. The patient presented to our Dermatology Department with large and painful coalescent cutaneous lesions on both lower extremities, fingertips and lips. Both clinical picture and disease history indicated systemic vasculitis that was previously unrecognized. Additional examinations revealed the following markers of inflammation: leukocytosis (11 G/I) with normal level of eosinophils, CRP > 0.6 mg/I, ESR 120 mm/h, and IgG 20 g/l, without any source of infection. Indirect immunofluorescence showed the absence of antineutrophil cytoplasmic antibodies (ANCA) on polynuclear leucocytes. A distinct fluorescence pattern observed in liver sinusoids was suggestive of the presence of some form of anti-granulocyte antibodies. Anti-nuclear antibodies (ANA) were detected in serum (1:1000) with homogenous and microgranular fluorescence pattern. Anti-extractable nuclear antigen panel (ENA), C3/C4 levels, anti-smooth muscle, anti-dsDNA and anti-glomerular basement membrane (GBM) antibodies were within normal limits. Lupus band test revealed IgA and IgG deposits at the dermis-epidermis junction. Histopathological examination of the skin showed calcification complicated by small vessel thrombosis, however, it was non-specific for vasculitis. Our case represents an example of calcyphylaxis associated with a widespread medial vascular calcification in a patient with end stage renal disease secondary to systemic lupus erythematosus. Although renal dialysis itself is known to trigger calcyphylaxis, there have been few reports in literature suggesting that collagen vascular disease may also contribute to this process.
钙过敏表现为血管钙沉积和皮肤坏死，出现在肾衰竭的终末期。晚期系统性结缔组织病，如红斑狼疮，尤其是伴有血管炎，导致肾衰竭和尿毒症，可能会引发钙过敏。我们报告一例50岁男性患者，自43岁起因慢性肾小球肾炎导致终末期肾病而需要长期腹膜透析。该患者因双下肢、指尖和嘴唇出现大片疼痛性融合性皮肤损害而就诊于我们的皮肤科。临床症状和病史均提示存在先前未被识别的系统性血管炎。进一步检查发现以下炎症指标：白细胞增多（11 G/I），嗜酸性粒细胞水平正常，CRP>0.6 mg/I，ESR 120 mm/h，IgG 20 g/l，且无任何感染源。间接免疫荧光显示多核白细胞上无抗中性粒细胞胞浆抗体（ANCA）。在肝血窦中观察到的独特荧光模式提示存在某种形式的抗粒细胞抗体。血清中检测到抗核抗体（ANA）（1:1000），呈均匀和微粒状荧光模式。抗可提取核抗原谱（ENA）、C3/C4水平、抗平滑肌、抗双链DNA和抗肾小球基底膜（GBM）抗体均在正常范围内。狼疮带试验显示真皮-表皮交界处有IgA和IgG沉积。皮肤组织病理学检查显示钙化并伴有小血管血栓形成，但这对血管炎不具有特异性。我们的病例代表了一例与系统性红斑狼疮继发终末期肾病患者广泛的中膜血管钙化相关的钙过敏。虽然已知肾透析本身会引发钙过敏，但文献中很少有报道表明胶原血管病也可能促成这一过程。
