A diabetic patient with finger gangrene

A 72-year-old male with type 2 diabetes and end-stage renal disease (ESRD) receiving hemodialysis (HD) for 10 months presented to hospital with one week of worsening pain, swelling, and bluish-black discoloration in the second and fourth right, and third left fingers. Preceding trauma to the digits had not occurred. Physical examination revealed dry gangrene in the digits (Fig. A), but no necrotic lesions in the lower limbs. An arteriovenous fistula (AVF) was created in his left forearm. Laboratory examinations showed fairly controlled diabetes (HbA1c 7.3%, post-prandial blood glucose 175 mg/dL), leukocytosis, and elevated C-reactive protein. Serological markers for auto-immune diseases and vasculitis syndromes, including anti-nuclear, rheumatoid factor, anti-Jo1, anti–double-stranded DNA, cytoplasmic–anti-neutrophil cytoplasmic (ANCA), and perinuclear–ANCA antibodies were all negative. A radiograph showed prominent calcification of the digital arteries in both hands (Fig. B). Computed tomography–angiography revealed only incomplete arterial occlusions in the upper limbs. What is your diagnosis for this patient?

The findings were consistent with calciphylaxis, a rare, life-threatening syndrome involving systemic calcification of the arteries. It typically affects patients with ESRD, with an estimated annualized incidence of 0.35% in the US, and less than 0.01% in Japan, which is lower than that of Western countries [,]. One-year mortality in patients with calciphylaxis and ESRD is 45 to 80%, which is supposed to be related to sepsis, the most common cause of death, originating from infected wounds []. Despite poorly controlled diabetes that often manifests as acral gangrene of the foot, the upper limbs seldom show necrotic lesions without specific conditions that include calciphylaxis, warfarin-induced skin necrosis, atherosclerotic vascular diseases, arterial thromboembolism, vasculitis, infectious ulceration, and steal syndrome following an AVF creation []. Known risk factors, other than ESRD, included diabetes and hyperparathyroidism, which were associated with our patient. Earlier studies described how patients treated with peritoneal dialysis (PD) had a higher incidence of calciphylaxis than those treated with HD [,]. Our patient initially received PD but an intractable tunnel infection led to a change to HD; the preceding influence of PD might have increased the risk for the onset of calciphylaxis. The detailed pathogenesis and radical treatments for calciphylaxis are still not established. Our patient was surgically treated by amputation of the second and fourth right fingers at the metacarpophalangeal joint level.

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

We have obtained written consent from the patient for publication of the report.

Sho Osonoi: Conceptualization, Data curation, Investigation, Visualization, Writing – original draft, Writing – review & editing. Kiho Hamaura: Conceptualization, Data curation, Investigation, Visualization, Writing – original draft, Writing – review & editing. Hiroshi Murakami: Conceptualization, Supervision, Writing – review & editing.