Epidermolysis bullosa

Itchiness or pruritus is a common symptom which generates a huge load to patients with EB. It consists of an uncomfortable sensation in the skin related to the desire of scratching, it is defined as subjective as each person can experience it differently.

In healthy people, if it is presented in acute form it can serve as a protection mechanism to harmful external agents (as, for example, irritant products for the skin or pathogenic microorganisms).

In people with EB, it presents as a transverse symptom, which can affect both intact skin and areas of skin with wounds. Generally, patients with more severe types of EB tend to have itching more frequently. It is usually one of the most difficult symptoms to treat, especially in dystrophic EB (DEB) and pruritic DEB (P-DEB).

In all cases, the itch produces the necessity of scratching. This scratching has a negative effect generating an increased damage in the skin and new injuries (blisters, irritation, reopening of already healed injuries in close areas, etc.) with more itchiness associated and higher infection risk. Although scratching gives an initial relief (which might last for about 15-25 minutes), it can later increase the itchiness sensation due to the presence of new wounds and, also, due to friction and scratching can stimulate the transmitters of the inflammatory response. In this way, a "vicious circle of itch-scratching-itch" is generated that is difficult to break. A fundamental part of itch treatment consists of trying to break this "vicious circle" (Figure 1).