Rare in Adults, IgA Vasculitis Presents Differently Than in Children, Analysis Shows

Immunoglobulin A vasculitis (IgA) is a small blood vessel vasculitis uncommon in adults, but 1 case demonstrates the importance of considering it as a differential diagnosis in adults with small vessel vasculitis, according to the results of a study published in Cureus.

In the case, a 65-year-old woman presented with a diffuse painful rash across her arms, back, and legs, for 12 days, according to the study results.

The symptoms associated with the rash were fatigue, migratory lower extremity joint pain, and swelling of the ankles and feet.

She had not traveled recently, nor did have any illnesses or contacts who were sick. The woman did have asthma and seasonal allergies, however.

She took a daily multivitamin and a turmeric supplement, which she recently discontinued.

The skin rash showed blanchable, edematous, palpable, red annular papules and plaques on the woman’s back and extremities. The rash was mostly present on the lower extremities. No active lesions were found in her mouth.

The individual’s blood work showed significant hypoalbuminemia, which is an elevated erythrocyte sedimentation rate of 7 mm/hour and a positive antistreptolysin O. The rapid strep tests were negative and there were no cardiopulmonary abnormalities, effusion, and infiltrates shown in a chest X-ray.

A urine test showed the presence of blood, ketones, and protein.

The erythrocyte sedimentation rate returned to normal when checked again 4 days later.

The individual received a biopsy showing that there was superficial perivascular inflammation comprising lymphocytes, neutrophils, and occasional eosinophils.

There was no evidence of active vascular destruction or fibrin deposition, but the findings did show that there was a suspicion of small-vessel vasculitis .A diagnosis of IgA vasculitis was made, and a treatment of an oral 40-mg prednisone taper was prescribed over 21 days.

About 2 weeks later, the woman reported that she was no longer fatigued and her joint pain and swelling had gone away. She had no new skin eruptions since beginning the treatment, and the previous lesions were only faintly visible.Additional labs were ordered at her 1-month follow-up. The treatment regimen was completed, and the individual’s symptoms resolved.

Investigators in this case found that adult-onset IgA vasculitis differs in clinical presentation, as well as treatment compared with the pediatric population.

In children, IgA vasculitis symptoms consist of abdominal pain, arthritis, and palpable purpura. The woman studied did not have abdominal pain but did have renal involvement, because of hematuria andproteinuria.

Investigators noted that even though the diagnosis was IgA vasculitis, small-vessel vasculitis could not be completely ruled out, because immunofluorescence staining was not performed.

Under direct immunofluorescence, IgA deposition was found in the blood vessel walls. However, the biopsy in this study did not demonstrate these findings.

Investigators assume that this was because of the length of time that the papule was present.

Biopsies should be performed within 48 hours of a lesion to display the histological findings. In this case, the papule was present for more than 48 hours.

Treatment usually is not warranted for IgA vasculitis. The individual in this case was given corticosteroids, because of how distressing and widespread the lesions were.

Reference

Wallace CE, Sharma A. Adult onset immunoglobulin A (IgA) vasculitis secondary to group A streptococcus infection. Cureus. 2022;14(4):e23987. doi:10.7759/cureus.23987