Early clinical presentations and progression of calciphylaxis

Abstract

These cases demonstrate that calciphylaxis may be clinically misdiagnosed due to ill-defined presentations, particularly in the early stages without the characteristic features of livedo racemosa and ulceration. However, recognition in the early stages is critical to implement timely treatment. As such, definitively diagnostic skin biopsy should be considered early in suspected cases to confirm the diagnosis of calciphylaxis and ensure prompt management of this lethal disease.

Calciphylaxis in the Setting of Hemodialysis, Liver Cirrhosis, and Warfarin Therapy for Atrial Fibrillation: An Argument for Alternative Anti-Embolic Therapy

“…Many case reports, case-control studies, and observational studies have been published describing the predisposing factors for calciphylaxis which include: dependence on dialysis for more than two years, female gender, white race, obesity, diabetes mellitus, hypercalcemia, hyperphosphatemia, hypoalbuminemia, autoimmune conditions (such as systemic lupus erythematosus and rheumatoid arthritis), recurrent hypotension, and warfarin therapy. Hypercoagulable states, such as Protein C and S deficiency, antithrombin III deficiency, and antiphospholipid antibody syndrome, are also associated with this condition [1,[6][7].…”

“…Once the diagnosis is established, the formulation of a treatment plan with an interdisciplinary approach, including experts in multiple fields, should be considered [7]. Treatment involves wound management, analgesia, potential sodium thiosulfate therapy, management of mineral bone disease, adjustment of dialysis, and modification of risk factors [6][7]. Elimination of risk factors is an important component of the management of patients with calciphylaxis.…”

Management of Cutaneous Calciphylaxis

“…Early surgical debridement in patients with CP is essential, as surgical debridement is associated with higher 6-month survival when compared to lack of debridement [18][19][20]. Local wound treatment regimens should involve removal of wound debris, application of nonadhesive wound dressings, and utilization of antiseptic or antimicrobial agents as needed [18].…”

Calciphylaxis — pathogenesis and clinical picture

“…It can also be assumed that the low frequency of calciphylaxis diagnosis is at least in part caused by calciphylaxis being mistakenly classified as treatment-resistant vasculitis. This is why correctly collected medical history (renal failure, dialysis treatment, primary and secondary hyperparathyroidism, hypervitaminosis D 3 ) is vital [9]. However, final diagnosis can only be made after collecting biopsy specimens of affected skin and proving the presence of typical histopathological changes.…”

“…Można także przypuszczać, że znikoma częstość rozpoznawania kalcyfilaksji jest przynajmniej częściowo spowodowana mylnym klasyfikowaniem kalcyfilaksji jako opornego na leczenie zapalenia naczyń. Dlatego też w procesie diagnostycznym istotną rolę odgrywa dobrze zebrany wywiad (niewydolność nerek, dializoterapia pierwotna i wtórna nadczynność przytarczyc hyperwitaminoza D 3 ) [9]. Jednak ostateczne rozpoznanie można postawić po wykonania bioptatów zajętej skóry oraz wykazania w nim typowych zmian histopatologicznych.…”