61571 Cryofibrinogenemia secondary to multiple myeloma presenting as digital necrosis

Document Type

Conference Proceeding

Publication Date

9-1-2025

Publication Title

J Am Acad Dermatol

Abstract

A sixty-nine-year-old white female with a history of coronary artery disease, breast cancer, and quiescent ulcerative colitis with associated pyoderma gangrenosum presented with acute on chronic ischemic fingers. She had experienced infrequent episodes of digital necrosis over the past year, necessitating outpatient surgical amputation at her right third and left second distal interphalangeal (DIP) joints. Prior evaluation was negative for hereditary thrombophilia, antiphospholipid syndrome, cryoglobulinemia, and arterial stenosis of the upper extremities. Despite empiric treatment with nitropaste, sildenafil, and nifedipine for suspected primary Raynaud’s phenomenon, she continued to exhibit episodic pink-white and blue-black color changes with severe pain involving her fingertips. Skin examination revealed retiform purpura on the left dorsomedial foot, a dusky blue patch on the left third fingertip, and distal amputations of the right third and left second fingers. A punch biopsy of the left great toe showed epidermal necrosis and underlying vascular thrombosis consistent with thrombotic vasculopathy. Direct immunofluorescence was negative. Expanded work-up including monoclonal protein serum testing revealed an elevated free light chain ratio (140.36) with significantly elevated kappa light chains (1010.6 mg/L). Retrospective immunohistochemical studies demonstrated positive kappa light chain staining within the vessels, while stains for Congo red and lambda were negative. Cryofibrinogen testing was positive. Hematology consultation and subsequent bone marrow biopsy confirmed multiple myeloma with 16% kappa-monocytic plasma cells. This case highlights a rare instance of cryofibrinogenemia secondary to multiple myeloma causing acral ischemia. Cryofibrinogenemia is an underrecognized disorder requiring a high index of suspicion to arrive at an accurate diagnosis.

Volume

93

First Page

AB31

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