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Kalziphylaxie

Ein wenig bekanntes, klinisch relevantes Krankheitsbild

Calciphylaxis

A less well-known, clinically relevant disease

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Zusammenfassung

Die Kalziphylaxie ist ein seltenes Krankheitsbild mit zumeist ungünstiger Prognose, welches mit zunehmender Häufigkeit diagnostiziert wird. Das klinische Bild der Kalziphylaxie ist geprägt von ausgedehnten ischämischen Ulzerationen und Nekrosen von Haut und Unterhaut. Histologisch liegen in den betroffenen Geweben typischerweise prominente Verkalkungen der kleinkalibrigen Gefäße vor. Aufgrund der ausgedehnten Wundflächen sowie aufgrund der häufig bestehenden Komorbiditäten sind Kalziphylaxiepatienten sehr anfällig für Infektionen und konsekutive septische Verläufe. In der vorliegenden Arbeit wird der Fall einer nierentransplantierten Patientin mit Vaskulitis geschildert, welche 4 Jahre nach Transplantation trotz guter Transplantatfunktion eine fulminante Kalziphylaxie im Bereich beider Oberschenkel entwickelte und an einer Sepsis verstarb. Die angesichts der Anamnese sowie des makroskopischen Befundes infrage kommenden Differenzialdiagnosen sowie das klinisch Prozedere werden in der Kasuistik detailliert dargestellt. Die weitere Arbeit gibt einen umfassenden Überblick über den aktuellen Kenntnisstand hinsichtlich Ätiopathogenese, Risikofaktoren, Diagnostik und klinischem Management der Kalziphylaxie.

Abstract

Calciphylaxis is a rare disease which has been increasingly reported in recent decades and has consequently shifted into the focus of clinical and scientific research. The clinical picture is characterized by extensive ischemic ulcerations of the skin and subcutis. Histologically, the small vessels in these lesions show prominent calcifications. Due to the extensive areas of ulceration and necrosis as well as frequently present comorbidities, patients with calciphylaxis are prone to infection and sepsis. In this work, we describe the case of a female kidney-transplant patient with vasculitis who, despite good graft function, developed a fulminant calciphylaxis of both thighs 4 years post transplantation and died of septic complications. The differential diagnoses as well as clinical procedures are described in detail in the case history. In the discussion, we give an overview of the current state of knowledge regarding the etiopathogenesis, risk factors, diagnostic measures and clinical management of calciphylaxis.

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Authors and Affiliations

  1. Abteilung für Nephropathologie, Pathologisches Institut, Universitätsklinikum Erlangen, Krankenhausstr. 12, 91054, Erlangen, Deutschland

    J.D. Strehl, A. Hartmann &  K. Amann

  2. Medizinische Klinik 1, RWTH Aachen, Aachen, Deutschland

    V. Brandenburg

  3. Medizinische Klinik 4, Universitätsklinikum Erlangen, Erlangen, Deutschland

    C. Forster & C. Willam

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  1. J.D. Strehl
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  2. V. Brandenburg
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  3. C. Forster
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  4. C. Willam
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  5. A. Hartmann
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  6. K. Amann
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Correspondence to K. Amann.

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Strehl, J., Brandenburg, V., Forster, C. et al. Kalziphylaxie. Pathologe 32, 250–256 (2011). https://doi.org/10.1007/s00292-010-1411-1

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  • DOI: https://doi.org/10.1007/s00292-010-1411-1

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