A Clinical and Histologic Study of 37 Cases of Immunoglobulin A-Associated Vasculitis

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Abstract
Immunoglobulin (Ig) A-associated vasculitis is commonly equated with the multiorgan systemic vasculitic syndrome Henoch-Schonlein purpura (HSP), which occurs predominantly in the pediatric age group. By natural language search of the databases of two outpatient dermatopathology practices, the authors selected for review 37 cases of IgA-associated vasculitis, 23 of which were associated with antecedent infection, most commonly of the upper respiratory tract. Criteria for a diagnosis of HSP were met in 15 cases, 13 of which were in the setting of prior infection. Lower extremity skin involvement was ubiquitous. A more widespread form of vasculitis was also seen, particularly in the setting of previous infection. Several of the patients with previous infection had underlying medical illnesses including rheumatoid arthritis, atopy, renal failure, lupus erythematosus, insulin dependent diabetes mellitus, autoimmune thyroid disease, and Wegener's granulomatosis. In those patients lacking an apparent microbial trigger, Sjogren's disease with anti-Ro antibodies and hypergammaglobulinemia, lupus erythematosus, inflammatory bowel disease, IgA paraproteinemia, bronchogenic and prostatic carcinoma, cryoglobulinemia, and lymphoma were uncovered. Regardless of whether an infectious stimulus was implicated, certain cofactors with the potential to enhance vascular injury were uncovered; these included anti-Ro antibodies, antineutrophil cytoplasmic antibody, diabetic microangiopathy, and a hyperviscosity state. In the infective group, a pustular vasculitis, defined as a neutrophilic vascular reaction in concert with epithelial pustulation, was seen in 81% of cases versus 33% in the non-infectious group (p = 0.02). The prototypic histomorphology in the noninfective group was one of a mild cell poor leukocytoclastic vasculitis; Vasculitis was of greater severity in patients with antecedent infection (p = 0.026). An infectious trigger, typically of mucosal origin, can frequently be identified in patients with cutaneous IgA-associated vasculitis, especially those with the symptom complex of HSP. The light microscopy appears to distinguish patients who have an infectious trigger from those who do not. IgA-associated vasculitis may be a clue to the presence of certain underlying disorders where there is immune dysregulation or enhanced susceptibility to immune complex entrapment.