Sausage on a string – two interesting case reports of Dense Deposit Disease

  • Dr Dheepa Senthil Kumar,*  
  • subalakshmi balasubramanian,  
  • Barathi Gunabooshanam,  
  • Dr Ponneyin Chelvi

Abstract

Dense deposit disease(DDD) is an ideal form of C3 glomerulopathy(C3G) ,with an estimated incidence of 2-3 people per million. DDD is defined by intramembranous C3 accumulation with absent or scanty immunoglobulin deposition and highly electron dense deposits visible by electron microscopy(EM). C3G is a unique and rational disease classification based on pathogenesis of dysregulated alternative complement pathway and is composed of DDD and C3 glomerulonephritis(C3GN). DDD is characterized by thickening of glomerular basement membrane by dense intramembranous deposits. Herein we present two cases of DDD diagnosed with a combined approach of clinical, pathological (light microscopy and electron microscopy) and laboratory investigations. Only EM assists definite distinction of DDD from C3 glomerulonephritis(C3GN).


Keywords

Dense Deposit Disease, Electron microscopy