Penile involvement of immunoglobulin a vasculitis/Henoch-Schönlein purpura

Abstract

Background: Immunoglobulin A vasculitis/Henoch-Schönlein purpura (IgAV/HSP) is a leukocytoclasia vasculitis of childhood, predominantly affecting the skin, joints, gastrointestinal tract, and kidneys. The involvement of the penis is rare.

Objective: We aimed to describe this rare manifestation of IgAV/HSP and to review the previous studies, including similar cases.

Methods: Clinical data were reviewed for two children of penile involvement of IgAV/HSP in our hospital. More clinical cases were retrieved from the databases of PubMed/MEDLINE and Scopus from database inception to February 1, 2020.

Results: We presented two boys aged three and five years both of whom had penile lesions after presenting with the typical rash of IgAV/HSP on lower extremities. The penile lesions improved entirely in a few days without treatment in one and with corticosteroid treatment in the other. The literature review revealed 12 articles describing 20 patients with penile involvement of IgAV/HSP. The penile findings were edema, erythema, ecchymosis, purpuric rash, edema, color change, stiffness of the shaft or prepuce, and tenderness. Penile lesions appeared before the purpuric rash of IgAV/HSP in three of 22 patients. The penile involvement could make the diagnosis challenging especially if the penile lesions appear before the typical rash of the disease. The lesions improved entirely in the short term in all patients; in five without treatment in fifteen after corticosteroid or immunosuppressive drug treatment.

Conclusions: It is important to raise awareness about this rare manifestation among health care providers. It is not clear whether corticosteroid treatment should be initiated for treatment since it seems as a self-limited feature. Treatment with corticosteroids could be considered in the treatment of selected cases especially with systemic involvement.

Keywords: Immunoglobulin a vasculitis; Penile involvement; Penis; Purpura.