Immunoglobulin A Vasculitis Masquerading as Bilateral Avascular Necrosis of the Hip

Nezam Altorok

doi:10.26717/bjstr.2020.26.004325

Abstract

IgA vasculitis is a small vessel vasculitis that usually presents acutely and resolves with supportive care. However, it can cause serious complications. The etiology of IgA vasculitis and the pathophysiology of atraumatic avascular necrosis of the hip remain largely unknown. Adult IgA vasculitis can rarely become complicated by avascular necrosis.

Highlights

Immunoglobulin A vasculitis (IgA vasculitis), formerly known as Henoch-Schönlein purpura (HSP), is a small vessel vasculitis that is mediated by immune complex deposition in the small vessels associated with activation of leukocytes, leaving IgA deposits in the vessel wall
We describe a unique case of IgA vasculitis first presented as acute bilateral hip pain followed by palpable purpura of the upper and lower extremities and abdominal pain
Avascular necrosis is a rare initial presentation of IgA vasculitis that has not been frequently reported in the literature

Summary

Introduction

Immunoglobulin A vasculitis (IgA vasculitis), formerly known as Henoch-Schönlein purpura (HSP), is a small vessel vasculitis that is mediated by immune complex deposition in the small vessels associated with activation of leukocytes, leaving IgA deposits in the vessel wall. We describe a unique case of IgA vasculitis first presented as acute bilateral hip pain followed by palpable purpura of the upper and lower extremities and abdominal pain. Our patient is a 43-year-old male with no significant past medical history, who presented with bilateral hip pain, predominantly on the right side. Direct immunofluorescence examination was negative for IgM and IgG It showed granular deposition of IgA and C3 within the walls of few superficial dermal vessels. Due to the severe symptoms and the MRI findings of subcortical fracture of the right hip, the patient underwent right total hip replacement (Figure 4) He was treated with a short course of prednisone 0.5mg / kg on a tapered dosing regimen for 1 month after the hip replacement. The new regimen was adequate to resolve his skin lesions

Discussion

Conclusion