Diagnosing Calciphylaxis: A Review With Emphasis on Histopathology.


Journal

The American Journal of dermatopathology
ISSN: 1533-0311
Titre abrégé: Am J Dermatopathol
Pays: United States
ID NLM: 7911005

Informations de publication

Date de publication:
Jul 2020
Historique:
entrez: 1 7 2020
pubmed: 1 7 2020
medline: 17 4 2021
Statut: ppublish

Résumé

Calciphylaxis is a cutaneous vasculopathy with high morbidity and mortality characterized by vascular intimal fibrosis, calcification, stenosis, thrombosis, and eventual tissue death due to ischemia. Histopathologic diagnosis is often difficult, frequently necessitating multiple tissues samples due to lack of specific histopathologic features and subtle changes on biopsies of early lesions. In this study, we review the reported clinical and histopathologic features of calciphylaxis, correlating them with relevant imaging, ancillary studies, and pathophysiology. Although many histopathologic changes seen in calciphylaxis are also reported in other conditions (eg, Mönckeberg sclerosis, lupus panniculitis, pancreatic panniculitis, and peripheral artery disease), calcification of subcutaneous small vessels, thrombosis and/or ischemic changes, pseudoxanthoma elasticum-like changes in the subcutis, and perieccrine calcification may serve as helpful clues. von Kossa and Alizarin red stains can assist in the identification of subtle calcification. Netlike calcification of the affected blood vessels on imaging further supports the diagnosis. Studies into the pathophysiology of calciphylaxis are ongoing and will hopefully facilitate the development of additional diagnostic adjuncts to increase sensitivity and specificity for the diagnosis of this disease.

Identifiants

pubmed: 32604207
doi: 10.1097/DAD.0000000000001526
pii: 00000372-202007000-00001
doi:

Types de publication

Journal Article Review

Langues

eng

Sous-ensembles de citation

IM

Pagination

471-480

Références

Fine A, Zacharias J. Calciphylaxis is usually non-ulcerating: risk factors, outcome and therapy. Kidney Int. 2002;61:2210–2217.
Weenig RH. Pathogenesis of calciphylaxis: Hans Selye to nuclear factor kappa-B. J Am Acad Dermatol. 2008;58:458–471.
Nigwekar SU, Zhao S, Wenger J, et al. A nationally representative study of calcific uremic arteriolopathy risk factors. J Am Soc Nephrol. 2016;27:3421–3429.
McCarthy JT, El-Azhary RA, Patzelt MT, et al. Survival, risk factors, and effect of treatment in 101 patients with calciphylaxis. Mayo Clin Proc. 2016;91:1384–1394.
Brandenburg VM, Kramann R, Rothe H, et al. Calcific uraemic arteriolopathy (calciphylaxis): data from a large nationwide registry. Nephrol Dial Transplant. 2017;32:126–132.
Selye H. The dermatologic implications of stress and calciphylaxis. J Invest Dermatol. 1962;39:259–275.
Winkelmann RK, Keating FR. Cutaneous vascular calcification, gangrene and hyperparathyroidism. Br J Dermatol. 1970;83:263–268.
Wilmer WA, Magro CM. Calciphylaxis: emerging concepts in prevention, diagnosis, and treatment. Semin Dial. 2002;15:172–186.
Coates T, Kirkland GS, Dymock RB, et al. Cutaneous necrosis from calcific uremic arteriolopathy. Am J Kidney Dis. 1998;32:384–391.
Nigwekar SU, Thadhani R, Brandenburg VM. Calciphylaxis. N Engl J Med. 2018;378:1704–1714.
James WD, Elston DM, Berger TG, eds. Andrews' Diseases of the Skin: Clinical Dermatology. 12th ed. Philadelphia, PA: Elsevier; 2016.
Bolognia JL, Schaffer JV, Cerroni L, eds. Dermatology. 4th ed. Philadelphia, PA: Elsevier; 2018.
Stompór T. An overview of the pathophysiology of vascular calcification in chronic kidney disease. Perit Dial Int. 2007;27(suppl 2):S215–S222.
Nigwekar SU, Jiramongkolchai P, Wunderer F, et al. Increased bone morphogenetic protein signaling in the cutaneous vasculature of patients with calciphylaxis. Am J Nephrol. 2017;46:429–438.
Mochel MC, Arakaki RY, Wang G, et al. Cutaneous calciphylaxis: a retrospective histopathologic evaluation. Am J Dermatopathol. 2013;35:582–586.
Nigwekar SU, Kroshinsky D, Nazarian RM, et al. Calciphylaxis: risk factors, diagnosis, and treatment. Am J Kidney Dis. 2015;66:133–146.
Brandenburg VM, Evenepoel P, Floege J, et al. Lack of evidence does not justify neglect: how can we address unmet medical needs in calciphylaxis? Nephrol Dial Transplant. 2016;31:1211–1219.
Shmidt E, Murthy NS, Knudsen JM, et al. Net-like pattern of calcification on plain soft-tissue radiographs in patients with calciphylaxis. J Am Acad Dermatol. 2012;67:1296–1301.
Halasz CL, Munger DP, Frimmer H, et al. Calciphylaxis: comparison of radiologic imaging and histopathology. J Am Acad Dermatol. 2017;77:241–246.e3.
Kröger K, Stang A, Kondratieva J, et al. Prevalence of peripheral arterial disease—results of the Heinz Nixdorf recall study. Eur J Epidemiol. 2006;21:279–285.
Lanzer P, Boehm M, Sorribas V, et al. Medial vascular calcification revisited: review and perspectives. Eur Heart J. 2014;35:1515–1525.
Williams EA, Moy AP, Cipriani NA, et al. Factors associated with false-negative pathologic diagnosis of calciphylaxis. J Cutan Pathol. 2018;46:16–25.
Nathoo RK, Harb JN, Auerbach J, et al. Pseudoxanthoma elasticum-like changes in nonuremic calciphylaxis: case series and brief review of a helpful diagnostic clue. J Cutan Pathol. 2017;44:1064–1069.
Ghosh T, Winchester DS, Davis MDP, et al. Early clinical presentations and progression of calciphylaxis. Int J Dermatol. 2017;56:856–861.
Chen TY, Lehman JS, Gibson LE, et al. Histopathology of calciphylaxis: cohort study with clinical correlations. Am J Dermatopathol. 2017;39:795–802.
McMullen ER, Lowe L, Fullen D, et al. Clinicopathologic features and calcium deposition patterns in calciphylaxis: comparison with peripheral artery disease, chronic stasis, and thrombotic vasculopathy. Abstract presented at USCAP Annual Meeting, Vancouver, Canada: University of Michigan, Ann Arbor, MI; 2018.
Nikko AP, Dunningan M, Cockerell CJ. Calciphylaxis with histologic changes of pseudoxanthoma elasticum. Am J Dermatopathol. 1996;18:396–399.
Penn LA, Brinster N. Calciphylaxis with pseudoxanthoma elasticum-like changes: a case series. J Cutan Pathol. 2018;45:118–121.
Fernandez KH, Liu V, Swick BL. Nonuremic calciphylaxis associated with histologic changes of pseudoxanthoma elasticum. Am J Dermatopathol. 2013;35:106–108.
Lewis KG, Lester BW, Pan TD, et al. Nephrogenic fibrosing dermopathy and calciphylaxis with pseudoxanthoma elasticum-like changes. J Cutan Pathol. 2006;33:695–700.
Kramann R, Brandenburg VM, Schurgers LJ, et al. Novel insights into osteogenesis and matrix remodelling associated with calcific uraemic arteriolopathy. Nephrol Dial Transplant. 2013;28:856–868.
Tian F, Patterson AT, Davick JJ, et al. The cutaneous expression of vitamin K-dependent and other osteogenic proteins in calciphylaxis stratified by clinical features and warfarin use: a case control study. J Am Acad Dermatol. 2016;75:840–842.e1.
Griethe W, Schmitt R, Jurgensen JS, et al. Bone morphogenic protein-4 expression in vascular lesions of calciphylaxis. J Nephrol. 2003;16:728–732.
Derwall M, Malhotra R, Lai CS, et al. Inhibition of bone morphogenetic protein signaling reduces vascular calcification and atherosclerosis. Arterioscler Thromb Vasc Biol. 2012;32:613–622.
Malhotra R, Burke MF, Martyn T, et al. Inhibition of bone morphogenetic protein signal transduction prevents the medial vascular calcification associated with matrix Gla protein deficiency. PLoS One. 2015;10:e0117098.
Morrell NW, Bloch DB, ten Dijke P, et al. Targeting BMP signalling in cardiovascular disease and anaemia. Nat Rev Cardiol. 2016;13:106–120.
Yao Y, Bennett BJ, Wang X, et al. Inhibition of bone morphogenetic proteins protects against atherosclerosis and vascular calcification. Circ Res. 2010;107:485–494.
Nigwekar SU, Nazarian RM. Cutaneous calcification in patients with kidney disease is not always calciphylaxis. Kidney Int. 2018;94:244–246.
Ishak R, Abbas O. Penicillamine revisited: historic overview and review of the clinical uses and cutaneous adverse effects. Am J Clin Dermatol. 2013;14:223–233.
Bercovitch L, Martin L, Chassaing N, et al. Acquired pseudoxanthoma elasticum presenting after liver transplantation. J Am Acad Dermatol. 2011;64:873–878.
Aessopos A, Savvides P, Stamatelos G, et al. Pseudoxanthoma elasticum-like skin lesions and angioid streaks in beta-thalassemia. Am J Hematol. 1992;41:159–164.
Baccarani-Contri M, Bacchelli B, Boraldi F, et al. Characterization of pseudoxanthoma elasticum-like lesions in the skin of patients with beta-thalassemia. J Am Acad Dermatol. 2001;44:33–39.
Bowen AR, Götting C, LeBoit PE, et al. Pseudoxanthoma elasticum-like fibers in the inflamed skin of patients without pseudoxanthoma elasticum. J Cutan Pathol. 2007;34:777–781.
Weenig RH, Sewell LD, Davis MDP, et al. Calciphylaxis: natural history, risk factor analysis, and outcome. J Am Acad Dermatol. 2007;56:569–579.

Auteurs

Eman Bahrani (E)

Resident Physician, Department of Dermatology, University of California, San Francisco, CA.

Ifeoma U Perkins (IU)

Dermatopathology Fellow, Departments of Pathology and Dermatology, University of California, San Francisco, CA; and.

Jeffrey P North (JP)

Assistant Professor, Departments of Pathology and Dermatology, University of California, San Francisco, CA.

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