Kalziphylaxie

Abstract

Calciphylaxis is a rare disease associated with thrombotic cutaneous ischemia and necrosis. Lesions are usually located on the lower extremities, buttocks and the abdomen. Calciphylaxis is recognized by medial calcification, inflammation and subintimal fibrosis of cutaneous arterioles. Calcification, thrombus formation and occlusion occur sequential before tissue necrosis. The disease is usually observed in patients with end-stage renal disease and hyperparathyroidism. In end-stage renal disease, an elevated parathyroid hormone level, hypercalcemia and hyperphosphatemia direct to vascular mineralization. Calciphylaxis affects about 4% of hemodialysis patients. The clinical syndrome is characterized by a high mortality rate. The most important measure is an active multidisciplinary management approach, with intention to wound care and prevention of sepsis.

Fibroblast involvement in soft connective tissue calcification

“…A typical example is represented by “calciphylaxis,” a rare disease in which a generalized calcification is associated with thrombotic cutaneous ischemia and necrosis, thus causing a mortality rate ranging from 60 to 80% due to wound infection, sepsis, and subsequent organ failure (Arseculeratne et al, 2006; Hoff and Homey, 2011). …”

<p>Management of patients with calciphylaxis: current perspectives</p>

“…11 Other causes of cutaneous vessel occlusion like cholesterin embolization, cryoglobulinemia or medication-induced ulcerations have to be differentiated, too. 12 In a case-control study of CP patients with concomitant renal disease, the authors found higher rates of hypercoagulability in the CP patients than in controls with same stages of renal diseases. 13 To differentiate other underlying diseases with the presentation of skin ulcers parameter for collagenoses or vasculitis, as well as cryoglobulines and antiphospholipid antibodies should be controlled.…”

“…13 To differentiate other underlying diseases with the presentation of skin ulcers parameter for collagenoses or vasculitis, as well as cryoglobulines and antiphospholipid antibodies should be controlled. 12 Taken together, there is some hint of a correlation between CP and hypercoagulability. 14 Calcium-phosphate levels, stage of renal disease, hypercoagulability and exclusion of differential diagnoses can mainly be performed via an orientating blood analysis.…”

“…38 However in a comprehensive review, the use of cinacalcet showed a reduction of parathyroid hormone within a period of 1-2 years, 39 so some authors only recommend parathyroidectomy in cases with no response to medication with cinacalcet. 12 A new substance (SNF472) to inhibit progressive calcification in patients with hemodialysis may be promising and future study results have to be awaited to assess its efficacy in CP patients (NCT02790073). The substance inhibits the development of ectopic calcification by blocking the formation and growth of hydroxyapatite crystal.…”