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Premature diagnosis of calciphylaxis without pathological indications finally diagnosed as cutaneous small-vessel

Hironori Nakamura, Mariko Anayama, Yasushi Makino

    Nihon Jinzo Gakkai Shi
    |December 15, 2018
    PubMed

    Insights

    This case study highlights a patient with diabetes and a history of heart conditions who developed severe skin lesions. Despite initial suspicion of calciphylaxis, the final diagnosis was cutaneous small-vessel vasculitis.

    Area of Science:

    • Dermatology
    • Nephrology
    • Cardiology

    Background:

    • Patient presented with a history of diabetes, myocardial infarction, and ventricular septal perforation.
    • Underwent hemodialysis and was on amiodarone treatment.
    • History of cardiac conditions and renal replacement therapy.

    Observation:

    • Presented with purpuric sores on lower limbs.
    • Skin biopsy revealed leukocytoclastic vasculitis, negative for immunofluorescence.
    • Lesions worsened despite topical treatment, raising suspicion for calciphylaxis.

    Findings:

    • Second biopsy confirmed leukocytoclastic vasculitis but lacked arterial calcification.
    • Autoantibody testing ruled out ANCA-associated vasculitis, cryoglobulinemia, and antiphospholipid syndrome.
    • Despite prednisolone treatment, the patient experienced systemic deterioration, succumbing to disseminated intravascular coagulation.

    Implications:

    • This case underscores the diagnostic challenges in differentiating calciphylaxis from other vasculitic syndromes.
    • Highlights the importance of comprehensive histopathological and serological evaluation in complex cases.
    • Emphasizes the poor prognosis associated with severe cutaneous small-vessel vasculitis in patients with multiple comorbidities.

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