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Navigating Diagnostic Uncertainty in a Case of Atypical Vasculitis
Background Vasculitides encompass a diverse group of rare inflammatory disorders that affect blood vessels, each presenting with distinct clinical and pathological characteristics. Over 30 types of vasculitides have been identified, differentiated by their patterns of vessel involvement, serological markers, and histopathological features. Accurate diagnosis is crucial for determining long-term survival and appropriate therapy, necessitating a thorough evaluation to classify the specific type of vasculitis and exclude other conditions that mimic it. Case Presentation We present a 59-year-old female who presented with oral mucositis, purpuric skin lesions, and bilateral cyanosis of the toes. Upon presentation, necrosis of the toes was identified, and the patient underwent ten-toe amputation. Despite the resolution of mucositis and purpuric lesions, she now presents with acute skin changes to her left index finger concerning for additional necrosis. An angiogram revealed arteriographic evidence of vasculitis in small tributaries, displaying a beaded appearance along the plantar surface of the foot. Histological examination was nonspecific, showing necrosis with inflammatory cell infiltration. A comprehensive rheumatologic workup was negative for antiphospholipid antibodies, ANCA, anti-DNA, ANA, CCP, MPO, RF, and Proteinase 3. The patient was started on steroids, which halted the progression of necrosis in her index finger. Despite an extensive serological and histopathological workup, no clear predisposing cause or diagnosis was established. The working diagnosis remained thromboangiitis obliterans, attributed to a remote history of smoking. Through her stay and discharge, the treatment team conducted patient-centered rounds by engaging in open dialogue with the patient and involving her physician acquaintances. Discussion With an elusive diagnosis, the type of vasculitis afflicting our patient remained unidentified. Her constellation of findings does not align with any known form of vasculitis, and the work-up provides no evidence to corroborate a specific pathology. Despite a leading diagnosis of thromboangiitis obliterans, empiric steroid therapy provided clinical benefit, which is not typical for the condition. Furthermore, the lack of a definitive diagnosis complicates prognostic assessments and the selection of concurrent immunosuppressive treatments. While our patient was equally frustrated at the lack of definitive answers regarding a diagnosis, the team's open communication fostered a relationship of trust, resulting in the patient's cooperation, thus enabling the team to stabilize our patient's symptoms in the setting of diagnostic uncertainty. Frequent updates and patient-centered rounds increased transparency about the goals of the patient and medical team. Our case demonstrates the importance of a systematic, evidence- based approach to vasculitis workup and establishing a therapeutic alliance with our patients.