Cutaneous IgA Vasculitis in Primary Sjögren Disease: Findings From a Medical Record-Based Review

This study aimed to describe clinical observations of patients presenting with palpable purpura, which is suspected to represent IgA vasculitis (IgAV) based on direct immunofluorescence (DIF), who were ultimately diagnosed with cutaneous vasculitis associated with primary Sjögren disease (SjD).

We retrospectively reviewed biopsy reports from a single center between 2014 and 2022 consistent with IgAV, identifying patients who were concurrently diagnosed with SjD. Data collected included clinical history, serologic findings, and skin biopsy results with DIF. A focused literature review was also performed to assess prior reports and to explore potential underlying mechanisms.

We reviewed 103 biopsy reports from a single center between 2014 and 2022 that were consistent with IgAV. Three female patients with SjD who developed palpable purpura, initially suspected to represent IgAV, were identified. This corresponds to a prevalence of 2.91% (95% confidence interval 0.61%-8.28%). All patients were seropositive for antinuclear antibodies and anti-SSA antibodies. In two of the three cases, cutaneous vasculitis was the first manifestation of SjD. In all patients, skin biopsy demonstrated small-vessel leukocytoclastic vasculitis, and DIF revealed perivascular IgA and C3 deposits without IgG deposits. The diagnosis was revised to cutaneous vasculitis associated with SjD following comprehensive clinical evaluation.

This case series suggests that IgAV may overlap with SjD. Clinicians should consider underlying SjD in patients presenting with features of IgAV, and histopathologic findings alone should not exclude the possibility of an underlying systemic autoimmune disease.