Skip to main content
Erschienen in: Der Hautarzt 7/2011

01.07.2011 | Leitthema

Kalziphylaxie

Pathogenese und Therapie

verfasst von: N.-P. Hoff, Prof. Dr. B. Homey

Erschienen in: Die Dermatologie | Ausgabe 7/2011

Einloggen, um Zugang zu erhalten

Zusammenfassung

Die Kalziphylaxie ist eine seltene Erkrankung, die von ausgedehnten ischämischen Ulzerationen und Nekrosen der Haut gekennzeichnet ist. Prädilektionsstellen sind hierbei die untere Extremität, Gesäß, Flanke und Bauchwand. Bei der Kalziphylaxie kommt es zur progredienten Kalzifizierung, subintimalen Fibrose und zur begleitenden Entzündung von Arteriolen der Kutis und Subkutis. Es kommt über die Sequenz der arteriolären Mediaverkalkung und kutaner thrombotischer Ischämie zur ulzerierenden Nekrose. Als assoziierte Erkrankungen lassen sich häufig eine terminale dialysepflichtige Niereninsuffizienz sowie ein Hyperparathyreoidismus nachweisen. Bei einer terminalen Niereninsuffizienz kommt es durch die begleitenden metabolischen Störungen wie Hyperphosphatämie, Hyperkalziämie und Hyperparathyreoidismus zur Kalzifizierungsneigung. Die Kalziphylaxie betrifft etwa 1–4% der Hämodialysepatienten. Aufgrund einer hohen Mortalitätsrate muss eine rasche interdisziplinäre Betreuung mit dem Ziel, die Wundheilung zu fördern und septische Krankheitsverläufe zu verhindern, erfolgen.
Literatur
1.
Zurück zum Zitat Adrogué HJ, Frazier MR, Zeluff B, Suki WN (1981) Systemic calciphylaxis revisited. Am J Nephrol 1:177–183PubMedCrossRef Adrogué HJ, Frazier MR, Zeluff B, Suki WN (1981) Systemic calciphylaxis revisited. Am J Nephrol 1:177–183PubMedCrossRef
2.
Zurück zum Zitat Bakhireva LN, Barrett-Connor EL, Laughlin GA, Kritz-Silverstein D (2005) Differences in association of bone mineral density with coronary artery calcification in men and women: the Rancho Bernardo Study. Menopause 12:691–698PubMedCrossRef Bakhireva LN, Barrett-Connor EL, Laughlin GA, Kritz-Silverstein D (2005) Differences in association of bone mineral density with coronary artery calcification in men and women: the Rancho Bernardo Study. Menopause 12:691–698PubMedCrossRef
3.
Zurück zum Zitat Bucay N, Sarosi I, Dunstan CR et al (1998) Osteoprotegerin-deficient mice develop early onset osteoporosis and arterial calcification. Genes Dev 12:1260–1268PubMedCrossRef Bucay N, Sarosi I, Dunstan CR et al (1998) Osteoprotegerin-deficient mice develop early onset osteoporosis and arterial calcification. Genes Dev 12:1260–1268PubMedCrossRef
4.
Zurück zum Zitat Budisavljevic MN, Cheek D, Ploth DW (1996) Calciphylaxis in chronic renal failure. J Am Soc Nephrol 7:978–982PubMed Budisavljevic MN, Cheek D, Ploth DW (1996) Calciphylaxis in chronic renal failure. J Am Soc Nephrol 7:978–982PubMed
5.
Zurück zum Zitat Brancaccio D, Biondi ML, Gallieni M et al (2005) Matrix GLA protein gene polymorphisms: clinical correlates and cardiovascular mortality in chronic kidney disease patients. Am J Nephrol 25:548–552PubMedCrossRef Brancaccio D, Biondi ML, Gallieni M et al (2005) Matrix GLA protein gene polymorphisms: clinical correlates and cardiovascular mortality in chronic kidney disease patients. Am J Nephrol 25:548–552PubMedCrossRef
6.
Zurück zum Zitat Chan YL, Mahony JF, Turner JJ, Posen S (1983) The vascular lesions associated with skin necrosis in renal disease. Br J Dermatol 109:85–95PubMedCrossRef Chan YL, Mahony JF, Turner JJ, Posen S (1983) The vascular lesions associated with skin necrosis in renal disease. Br J Dermatol 109:85–95PubMedCrossRef
7.
Zurück zum Zitat Cozzolino M, Brandenburg V (2010) Warfarin: to use or not to use in chronic kidney disease patients? J Nephrol 23:648–652PubMed Cozzolino M, Brandenburg V (2010) Warfarin: to use or not to use in chronic kidney disease patients? J Nephrol 23:648–652PubMed
8.
Zurück zum Zitat Fine A, Fleming S, Leslie W (1995) Calciphylaxis presenting with calf pain and plaques in four continuous ambulatory peritoneal dialysis patients and in one predialysis patient. Am J Kidney Dis 25:498–502PubMedCrossRef Fine A, Fleming S, Leslie W (1995) Calciphylaxis presenting with calf pain and plaques in four continuous ambulatory peritoneal dialysis patients and in one predialysis patient. Am J Kidney Dis 25:498–502PubMedCrossRef
9.
Zurück zum Zitat Ketteler M, Bongartz P, Westenfeld R et al (2003) Association of low fetuin-A (AHSG) concentrations in serum with cardiovascular mortality in patients on dialysis: a cross-sectional study. Lancet 361:827–833PubMedCrossRef Ketteler M, Bongartz P, Westenfeld R et al (2003) Association of low fetuin-A (AHSG) concentrations in serum with cardiovascular mortality in patients on dialysis: a cross-sectional study. Lancet 361:827–833PubMedCrossRef
10.
Zurück zum Zitat Khoruts A, Stahnke L, McClain CJ et al (1991) Circulating tumor necrosis factor, interleukin-1 and interleukin-6 concentrations in chronic alcoholic patients. Hepatology 13:267–276PubMedCrossRef Khoruts A, Stahnke L, McClain CJ et al (1991) Circulating tumor necrosis factor, interleukin-1 and interleukin-6 concentrations in chronic alcoholic patients. Hepatology 13:267–276PubMedCrossRef
11.
Zurück zum Zitat Lang N, Davie R, Whitworth C (2004) Fatal calcific uraemic arteriolopathy (CUA): a case report and review of the literature. Scott Med J 49:108–111PubMed Lang N, Davie R, Whitworth C (2004) Fatal calcific uraemic arteriolopathy (CUA): a case report and review of the literature. Scott Med J 49:108–111PubMed
12.
Zurück zum Zitat Lal G, Nowell AG, Liao J et al (2009) Determinants of survival in patients with calciphylaxis: a multivariate analysis. Surgery 146:1028–1034PubMedCrossRef Lal G, Nowell AG, Liao J et al (2009) Determinants of survival in patients with calciphylaxis: a multivariate analysis. Surgery 146:1028–1034PubMedCrossRef
13.
Zurück zum Zitat London GM, Marty C, Marchais SJ et al (2004) Arterial calcifications and bone histomorphometry in end-stage renal disease. J Am Soc Nephrol 15:1943–1951PubMedCrossRef London GM, Marty C, Marchais SJ et al (2004) Arterial calcifications and bone histomorphometry in end-stage renal disease. J Am Soc Nephrol 15:1943–1951PubMedCrossRef
14.
Zurück zum Zitat Lue C, Boulware DW, Sanders PW et al (1996) Calciphylaxis mimicking skin lesions of connective tissue diseases. South Med J 89:1099–1100PubMedCrossRef Lue C, Boulware DW, Sanders PW et al (1996) Calciphylaxis mimicking skin lesions of connective tissue diseases. South Med J 89:1099–1100PubMedCrossRef
15.
Zurück zum Zitat Mehta RL, Scott G, Sloand JA, Francis CW (1990) Skin necrosis associated with acquired protein C deficiency in patients with renal failure and calciphylaxis. Am J Med 88:252–257PubMedCrossRef Mehta RL, Scott G, Sloand JA, Francis CW (1990) Skin necrosis associated with acquired protein C deficiency in patients with renal failure and calciphylaxis. Am J Med 88:252–257PubMedCrossRef
16.
Zurück zum Zitat Mohammed IA, Sekar V, Bubtana AJ et al (2008) Proximal calciphylaxis treated with calcimimetic „Cinacalcet“. Nephrol Dial Transplant 23:387–389PubMedCrossRef Mohammed IA, Sekar V, Bubtana AJ et al (2008) Proximal calciphylaxis treated with calcimimetic „Cinacalcet“. Nephrol Dial Transplant 23:387–389PubMedCrossRef
17.
Zurück zum Zitat Monney P, Nguyen QV, Perroud H, Descombes E (2004) Rapid improvement of calciphylaxis after intravenous pamidronate therapy in a patient with chronic renal failure. Nephrol Dial Transplant 19:2130–2132PubMedCrossRef Monney P, Nguyen QV, Perroud H, Descombes E (2004) Rapid improvement of calciphylaxis after intravenous pamidronate therapy in a patient with chronic renal failure. Nephrol Dial Transplant 19:2130–2132PubMedCrossRef
18.
Zurück zum Zitat Nordfors L, Lönnqvist F, Heimbürger O et al (1998) Low leptin gene expression and hyperleptinemia in chronic renal failure. Kidney Int 54:1267–1275PubMedCrossRef Nordfors L, Lönnqvist F, Heimbürger O et al (1998) Low leptin gene expression and hyperleptinemia in chronic renal failure. Kidney Int 54:1267–1275PubMedCrossRef
19.
Zurück zum Zitat Podymow T, Wherrett C, Burns KD (2001) Hyperbaric oxygen in the treatment of calciphylaxis: a case series. Nephrol Dial Transplant 16:2176–2180PubMedCrossRef Podymow T, Wherrett C, Burns KD (2001) Hyperbaric oxygen in the treatment of calciphylaxis: a case series. Nephrol Dial Transplant 16:2176–2180PubMedCrossRef
20.
Zurück zum Zitat Reynolds JL, Joannides AJ, Skepper JN et al (2004) Human vascular smooth muscle cells undergo vesicle-mediated calcification in response to changes in extracellular calcium and phosphate concentrations: a potential mechanism for accelerated vascular calcification in ESRD. J Am Soc Nephrol 15:2857–2867PubMedCrossRef Reynolds JL, Joannides AJ, Skepper JN et al (2004) Human vascular smooth muscle cells undergo vesicle-mediated calcification in response to changes in extracellular calcium and phosphate concentrations: a potential mechanism for accelerated vascular calcification in ESRD. J Am Soc Nephrol 15:2857–2867PubMedCrossRef
21.
Zurück zum Zitat Sankarasubbaiyan S, Scott G, Holley JL (1998) Cryofibrinogenemia: an addition to the differential diagnosis of calciphylaxis in end-stage renal disease. Am J Kidney Dis 32:494–498PubMedCrossRef Sankarasubbaiyan S, Scott G, Holley JL (1998) Cryofibrinogenemia: an addition to the differential diagnosis of calciphylaxis in end-stage renal disease. Am J Kidney Dis 32:494–498PubMedCrossRef
22.
Zurück zum Zitat Schlieper G, Brandenburg V, Ketteler M, Floege J (2009) Sodium thiosulfate in the treatment of calcific uremic arteriolopathy. Nat Rev Nephrol 5:539–543PubMedCrossRef Schlieper G, Brandenburg V, Ketteler M, Floege J (2009) Sodium thiosulfate in the treatment of calcific uremic arteriolopathy. Nat Rev Nephrol 5:539–543PubMedCrossRef
23.
Zurück zum Zitat Selye H (1962) Calciphlaxis. University of Chicago Press, Chicago, S 1 Selye H (1962) Calciphlaxis. University of Chicago Press, Chicago, S 1
24.
Zurück zum Zitat Shiraishi N, Kitamura K, Miyoshi T et al (2006) Successful treatment of a patient with severe calcific uremic arteriolopathy (calciphylaxis) by etidronate disodium. Am J Kidney Dis 48:151–154PubMedCrossRef Shiraishi N, Kitamura K, Miyoshi T et al (2006) Successful treatment of a patient with severe calcific uremic arteriolopathy (calciphylaxis) by etidronate disodium. Am J Kidney Dis 48:151–154PubMedCrossRef
25.
Zurück zum Zitat Tintut Y, Patel J, Parhami F, Demer LL (2000) Tumor necrosis factor-alpha promotes in vitro calcification of vascular cells via the cAMP pathway. Circulation 102:2636–2642PubMed Tintut Y, Patel J, Parhami F, Demer LL (2000) Tumor necrosis factor-alpha promotes in vitro calcification of vascular cells via the cAMP pathway. Circulation 102:2636–2642PubMed
26.
Zurück zum Zitat Tittelbach J, Graefe T, Wollina U (2001) Painful ulcers in calciphylaxis – combined treatment with maggot therapy and oral pentoxyfillin. J Dermatol Treat 12:211–214CrossRef Tittelbach J, Graefe T, Wollina U (2001) Painful ulcers in calciphylaxis – combined treatment with maggot therapy and oral pentoxyfillin. J Dermatol Treat 12:211–214CrossRef
27.
Zurück zum Zitat Vargemezis V, Liakopoulos V, Kriki P et al (2010) Pivotal role of paricalcitol in the treatment of calcific uremic arteriolopathy in the presence of a parathyroid adenoma. Am J Kidney Dis 55:144–147PubMedCrossRef Vargemezis V, Liakopoulos V, Kriki P et al (2010) Pivotal role of paricalcitol in the treatment of calcific uremic arteriolopathy in the presence of a parathyroid adenoma. Am J Kidney Dis 55:144–147PubMedCrossRef
28.
Zurück zum Zitat Weenig RH, Hewell LD, Davis MD et al (2007) Calciphlaxis: natural history, risk factor analysis, and outcome. J Am Acad Dermatol 56:569–579PubMedCrossRef Weenig RH, Hewell LD, Davis MD et al (2007) Calciphlaxis: natural history, risk factor analysis, and outcome. J Am Acad Dermatol 56:569–579PubMedCrossRef
29.
Zurück zum Zitat Weenig RH (2008) Pathogenesis of calciphylaxis: Hans Selye to nuclear factor kappa-B. J Am Acad Dermatol 58:458–471PubMedCrossRef Weenig RH (2008) Pathogenesis of calciphylaxis: Hans Selye to nuclear factor kappa-B. J Am Acad Dermatol 58:458–471PubMedCrossRef
31.
Zurück zum Zitat Zebboudj AF, Shin V, Boström K (2003) Matrix GLA protein and BMP-2 regulate osteoinduction in calcifying vascular cells. J Cell Biochem 90:756–765PubMedCrossRef Zebboudj AF, Shin V, Boström K (2003) Matrix GLA protein and BMP-2 regulate osteoinduction in calcifying vascular cells. J Cell Biochem 90:756–765PubMedCrossRef
Metadaten
Titel
Kalziphylaxie
Pathogenese und Therapie
verfasst von
N.-P. Hoff
Prof. Dr. B. Homey
Publikationsdatum
01.07.2011
Verlag
Springer-Verlag
Erschienen in
Die Dermatologie / Ausgabe 7/2011
Print ISSN: 2731-7005
Elektronische ISSN: 2731-7013
DOI
https://doi.org/10.1007/s00105-010-2111-8

Weitere Artikel der Ausgabe 7/2011

Der Hautarzt 7/2011 Zur Ausgabe

Einführung zum Thema

Wundheilung

Leitthema

Medikamente

CME Weiterbildung · Zertifizierte Fortbildung

Elektrochemotherapie von Hauttumoren

Leitlinien kompakt für die Dermatologie

Mit medbee Pocketcards sicher entscheiden.

Seit 2022 gehört die medbee GmbH zum Springer Medizin Verlag

Update Dermatologie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.