Journal of Vasculitis

Background: Thrombotic vasculitis is an uncommon cutaneous vasculopathy characterized by intravascular thrombosis where vessel wall inflammation precedes thrombosis. Terminology and classification are inconsistent and there are no standard treatment guidelines. Management is difficult due to overlap with other systemic vasculitis, calciphylaxis and other thrombotic and inflammatory disorders. This had led to uncertainty regarding the role and duration of anticoagulation. Cases series: We present a case series of three patients at the University of New Mexico with biopsy proven thrombotic vasculitis, supported by clinical and histopathologic images which demonstrate key diagnostic features. Presentations included painful ulcerations of the lower extremities and atypical cutaneous rashes involving the truncal region. Evaluation was performed to assess for autoimmune disease, hypercoagulable disorders, infections and malignancy. Two patients were found to have lupus-like inhibitor positivity and were managed with long term warfarin therapy, while one patient achieved durable clinical remission on a direct oral anticoagulant. All patients showed clinical improvement or resolution of lesions following initiation of systemic anticoagulation and were managed with multidisciplinary involvement from dermatology, hematology and rheumatology. Discussion: This case series highlights the clinical variability and management challenges of thrombotic vasculitis encountered in practice. Despite shared histopathologic findings, our patients differed in their presentation, risk factors and anticoagulation strategies. Skin biopsy was essential for diagnosis and laboratory evaluation helped guide treatment decisions. Anticoagulation was used in all cases with clinical improvement, particularly in patients with suspected hypercoagulable states, but decisions regarding agent selection and duration were individualized. Larger studies are needed to better define patient selection for anticoagulation and to guide clinical decision making.