Epidermolysis bullosa

The urinary system can be affected in many ways in people with EB. Blistering and narrowing of the urinary tract has been described, which can lead to a build-up of urine and damage to the kidney. This phenomenon is called hydronephrosis. 

On the other hand, the repetitive skin infections suffered by these people let the entrance of pathogens. There is a well-known relation between the infection by the Streptococcus pyogenes bacteria and the later inflammation of the kidney called post-streptococcal glomerulonephritis. 

 

Deposits of amyloid protein (called amyloidosis) have also been described in the renal glomerulus, which makes it difficult for the kidney to function and favors its inflammation and fibrosis. The mechanism causing the amyloidosis is unknown, but it has been associated with higher frequency to the dystrophic type. 

The renal affectations can cause a chronic renal insufficiency, even at an early age. It is recommended to control the renal functioning with certain periodicity in order to detect early on the possible renal manifestations before the damage is irreversible. 

In relation to the urinary and reproductive tract, some complications can appear on the penis as scars on the gland which close the exit (the urethral meatus), causing phimosis and, sometimes, the narrowing of the urethra, which needs of surgery. Regarding the external female genitalia, some cases of partial fusion of the vaginal labia and the narrowing of the vaginal orifice have been described. The complete physical and periodic exploration of the whole body surface allows to detect these complications.