Sir,

Calciphylaxis is a rare and highly morbid disease, also known as calcifying uremic arteriolopathy, occurring frequently in chronic kidney disease patients treated with dialysis. Other risk factors are mainly the female sex, diabetes, obesity, autoimmune diseases, liver disease, hypercoagulable states, and the use of certain drugs such as corticosteroids and methotrexate [1,2]. Its pathogenesis is complex and incompletely understood [3]. The diagnosis is primarily clinical. It is manifested by livedo and painful erythematous or purpuric lesions in the early stage. The evolution is toward the formation of nodules or indurated plaques, hemorrhagic bullae, and necrotic ulcers, which are preferentially located in the fat-rich areas (trunk, breasts, flanks, buttocks, and proximal lower limbs) [2,3]. Calciphylaxis should be considered when faced with an atypical presentation of vasculitis in a patient with renal failure [4]. There is often a significant inflammatory syndrome. Hypercalcemia and hyperparathyroidism are inconstant [4,5]. Perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA), cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA), and anti-neutrophil antibodies titers may be measured to exclude vasculitis [6]. Histology remains the gold standard for definitive diagnosis, especially in atypical cases, and shows calcification of the vascular wall, intraluminal microthrombosis, and fibrointimal hyperplasia of the dermal and subcutaneous arterioles [1,2]. Calcium deposits may be detected by von Kossa silver staining and Alizarin red staining [2]. Management is multidisciplinary and still uncodified, and is based on local and general measures: increased frequency or length of hemodialysis sessions, hyperbaric oxygen therapy, pain management, local care, and treatment of thrombosis and calcifications, notably with sodium thiosulfate [2–5]. The prognosis, both in terms of morbidity and survival rate at one year, is poor, and death is mainly related to septic complications [5,6].

Herein, we report the case of a 31-year-old male with chronic hemodialysis present for six years due to nephroangiosclerosis. For six months, he had extensive, painful skin lesions on the lower limbs evolving in a context of altered general condition. An examination revealed a ramified livedo, multiple, purpuric, ecchymotic, indurated, and highly painful patches, some of which were centered by ulcerations with a necrotic surface, predominantly on the thighs (Figs. 1a and 1b). Biologically, normal blood calcium levels, hyperphosphatemia, hyperparathyroidism, a major inflammatory syndrome, and a negative immunological work-up (AAN, ANCA) were noted. A skin biopsy was performed, showing calcifications in the vascular walls, as evidenced by von Kossa silver staining. The patient received local care and analgesics with an increase in the frequency of dialysis. He died several days later of septic shock.

Figure 1: (a) Ramified livedo with purpuric and ecchymotic indurated patches on the thigh.(b) Ulcerated, ecchymotic plaque on the calf.

REFERENCES

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