The link between vasculitis and malignancy is not fully understood; however, several mechanisms have been introduced to explain the possible association, including immune complex deposition, issues with clearance of immune complexes, and autoimmune cross-reactivity. In cases of malignancy, immune complexes with tumor antigens can form and deposit in small vessels, potentially triggering a complement cascade. Vascular injury to the vessels is a result of the inflammatory cascade attracting neutrophils, and the release of lysosomal enzymes resulting in fibrinoid necrosis. Vascular injury appears to be more consistent in the lower extremities, most likely as a result of increased turbulence and venous pressure in these regions. 4 Additionally, the production of antibodies against the tumor cells can cause cross-reactivity with endothelial cells, further contributing to vascular injury. 5
Although granulomatosis with polyangiitis( GPA) can present with recurrent sinusitis, epistaxis, and cutaneous lesions, several features in this case argue against isolated GPA. Radiographically, GPA typically produces cavitary nodules, airway-centered granulomatous inflammation, or diffuse lung involvement; these findings were absent in our patient’ s imaging studies. Renal involvement, which is commonly seen in GPA, was not present in this patient, as renal function remained normal with no hematuria or proteinuria. While MPO-Antineutrophil Cytoplasmic Antibodies( ANCA) positivity can occur in rare cases of limited GPA without kidney involvement, the skin biopsy in our patient revealed LCV rather than granulomatous inflammation. 6 These findings support a paraneoplastic etiology rather than isolated GPA.
Approximately 58 % of patients with LCV experience systemic symptoms, such as low-grade fever, malaise, weight loss, myalgia, and most commonly arthralgia. Less common findings may include renal, gastrointestinal, pulmonary, and neurological symptoms. 7 In the management of palpable purpura, the gold standard for diagnosis of LCV
is skin biopsy. Once confirmed, it is essential to rule out underlying conditions such as malignancy, sepsis, infection, or adverse drug effects( e. g., nonsteroidal anti-inflammatory drugs and diuretics) before proceeding with immunological screening panels. Systemic symptoms tend not to be the primary complaint in autoimmune diseases, such as rheumatoid arthritis or Sjögren syndrome, compared to underlying malignancy. 8
When pursuing management strategies for LCV, the underlying cause holds significant value. In drug-induced LCV, discontinuing the offending medication is crucial; regarding infectious agents, treating the associated infection typically leads to resolution of vasculitis. In malignancy-associated vasculitis, addressing the underlying malignancy often results in improvement of vasculitis symptoms, as illustrated in our case of a patient with right lung adenocarcinoma. 8 The initial treatment of vasculitis in our case consisted of RTX; however, it required subsequent colchicine therapy when the vasculitis did not completely subside.
FIGURE 1
CONCLUSION
This case underscores the critical association between LCV and solid organ malignancies, particularly lung adenocarcinoma. LCV is commonly linked to infection, medication, and autoimmunity, and its emergence in relation to paraneoplastic manifestation remains underrecognized. The patient’ s presentation of systemic symptoms( recurrent sinusitis, unintentional weight loss, bilateral purpuric lower extremity rash) combined with a history of underlying malignancy, prompted further investigation of recurrent and underlying malignancy. While the patient’ s recurrent sinusitis initially broadened the differential to include GPA, the lack of granulomatous disease on biopsy, renal studies within normal limits, and biopsy confirming leukocytoclastic vasculitis made the diagnosis unlikely. This case highlights the importance of considering occult malignancy in patients presenting with unexplained vasculitis. The recognition of vasculitis as a potential complication of malignancy can aid in the timely diagnosis of malignancy as well as guide further oncologic evaluation for recurrent malignancy.
Cutaneous manifestation showing non-blanching maculopapular lesions on the right lower extremity.
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