Scrotal involvement in childhood immunoglobulin A associated vasculitis

Abstract

Aim: The aim of this study is to evaluate the demographic and clinic findings in immunoglobulin A-associated vasculitis (IgAV) patients with scrotal involvement and also to determine predictive factors for assessing the development of scrotal involvement. Materiel and Methods: The medical records of 181 boys who were diagnosed with IgAV in the Pediatric Rheumatology Clinic of our center between September 2015-January 2021 were evaluated retrospectively. Results: A total number of 181 boys with IgAV included in the study. Twenty-seven (14.9%) of the 181 boys with IgAV had scrotal involvement. Among the scrotal-involved patients, 160 boys (88.4%) had scrotal swelling, 120 (66.3%) had erythema and 145 (80.1%) had scrotal pain or tenderness. Scrotal involvement was the first symptom of IgAV in one patient. CRP and WBC were significantly higher in the scrotal-involved group compared to the non-involved group (p=0.018, p=0.04, respectively). There were no significant differences in clinical findings and organ involvements between two groups. On ultrasonography, bilateral scrotal soft tissue thickening was observed in all patients. In 130 of the patients (71.8%), the epididymis was swollen with increased vascularity. The size, echogenicity and vascularity of the testicles were within normal limits. Eighty-seven of the patients (48%) were given steroids, 22 of them (11.7%) nonsteroidal anti-inflammatory drug. Conclusion: Scrotal involvement in boys with IgAV is not rare, it should be considered in the differential diagnosis in patients with scrotal pain, swelling and erythema. In addition, inflammatory markers may be higher in patients with scrotal involvement.

Keywords

• Immunoglobulin A-associated vasculitis
• Scrotal involvement
• MEFV
• Children

References

1. References 1. Ozen S, Sag E. Childhood vasculitis..Rheumatology (Oxford). 2020;59(Suppl 3):95-100. 2. Leung AKC, Barankin B, Leong KF. Henoch-Schonlein Purpura in Children: An Updated Review..Curr Pediatr Rev. 2020;16(4):265-76.
2. 3. Reamy BV, Williams PM, Lindsay TJ. Henoch–Schönlein purpura. Am Fam Physician 2009;80 (7):697–704.
3. 4. Dolezalová P, Telekesová P, Nemcová D et al. Incidence of vasculitis in children in the Czech Republic: 2-year prospective epidemiology survey. J. Rheumatol. 2004;31(11):2295–9.
4. 5. Weiss PF, Klink AJ, Luan X, Feudtner C. Temporal association of Streptococcus, Staphylococcus, and parainfluanza pediatric hospitalizations and hospitalized cases of Henoch-Schönlein purpura. J. Rheumatol. 2010;37(12):2587–94.
5. 6. Watanabe T. Henoch-Schönlein purpura following influenza vaccinations during the pandemic of influenza A (H1N1). Pediatr. Nephrol. 2011; 25(5):795–8.
6. 7. Ozen S, Pistorio A, Iusan SM et al. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II. Final classicication criteria. Ann. Rheum. Dis. 2010;69(5): 798–806.
7. 8. Ma Y, Zhang S, Chen J, et al. Henoch-Schonlein Purpura With Scrotal Involvement: A Case Report and Literature Review. J Pediatr Hematol Oncol. 2021 Apr 21. doi: 10.1097/MPH.0000000000002161.
8. 9. Allen DM, Diamond LK, Howell DA. Anaphylactoid purpura in children (Schonlein-Henoch syndrome): review with a follow-up of the renal complications. J Dis Child. 1960; 99:833-54.

9. 10. Clark WR, Kramer SA. Henoch-Schönlein purpura and the acute scrotum. J Pediatr Surg. 1986; 21(11):991-2.
10. 11. Hardoff D, Jaffe M. Recurrent episodes of testicular swelling preceding Henoch-Schönlein purpura by 11 months. Eur J Pediatr. 1987;146(6):613–4.
11. 12. O'Regan S, Robitaille P. Orchitis mimicking testicular torsion in Henoch-Schönlein's purpura. J Urol. 1981;126(6):834-5.
12. 13. Huang LH, Yeung CY, Shyur SD, et al. Diagnosis of Henoch-Schönlein purpura by sonography and radionuclear scanning in a child presenting with bilateral acute scrotum. J Microbiol Immunol Infect. 2004;37(3):192-5.
13. 14. Ben-Sira L, Laor T. Severe scrotal pain in boys with Henoch-Schonlein purpura: incidence and sonography. Pediatr Radiol. 2000;30(2):125-8.
14. 15. Tabel Y, Inanc FC, Dogan DG, et al. Clinical features of children with Henoch-Schonlein purpura: risk factors associated with renal involvement. Iran J Kidney Dis. 2012;6(4):269-74.
15. 16. Ha T-S, Lee J-S. Scrotal involvement in childhood Henoch Schönlein purpura. Acta Paediatr. 2007;96(4):552–5.
16. 17. Bayram C, Demircin G, Erdoğan O, et al. Prevalence of MEFV gene mutations and their clinical correlations in Turkish children with Henoch-Schonlein purpura. Acta Paediatr. 2011;100(5):745-9.