[Calciphylaxis. A less well-known, clinically relevant disease].
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.
Abteilung für Nephropathologie, Pathologisches Institut, Universitätsklinikum Erlangen, Krankenhausstr. 12, 91054, Erlangen.
J D
JD
Strehl
V
V
Brandenburg
C
C
Forster
C
C
Willam
A
A
Hartmann
K
K
Amann
D000368:Aged; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000473:pathology / Q000628:therapy; D001158:Arteries / Q000473:pathology; D002115:Calciphylaxis / Q000209:etiology / Q000473:pathology* / Q000628:therapy; D018450:Disease Progression; D004359:Drug Therapy, Combination; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use; D007668:Kidney / Q000473:pathology; D007676:Kidney Failure, Chronic / Q000473:pathology / Q000601:surgery / Q000628:therapy; D016030:Kidney Transplantation; D009102:Multiple Organ Failure / Q000473:pathology; D011183:Postoperative Complications / Q000209:etiology / Q000473:pathology* / Q000628:therapy; D006435:Renal Dialysis; D012307:Risk Factors; D012772:Shock, Septic / Q000209:etiology / Q000473:pathology / Q000628:therapy; D012867:Skin / Q000473:pathology
D000900:Anti-Bacterial Agents; D007166:Immunosuppressive Agents