Surgical treatment and management of cutaneous squamous cell carcinoma in patients with dystrophic epidermolysis bullosa - a case report.

Abstract

Epidermolysis bullosa (EB) is a rare inherited disease which is characterized by blisters on the skin and mucous membranes. Some forms of EB are associated with a risk of squamous cell carcinoma (SCC) development, which, unlike in the general population, is formed at a young age. SCC is the most common cause of death in patients with a dystrophic form. It is necessary to examine chronic and non-healing wounds for an increased risk of SCC. The basic treatment consists of surgical excision of the tumor site with a wide margin into healthy tissue. The surgical wound can be healed by secondary intention to prevent further trauma of the patient. The radicality of the excision is influenced by the location of the tumor. On the body, it is considerably limited by the surrounding tissue; on the limb, it is necessary to consider its amputation. In case of dissemination of the disease, it is important to approach patients individually and discuss other treatment options, including palliative care, within the national EB Center. The therapy is focused on pain treatment, remedial surgical dressings and psychological support with an emphasis on maintaining the quality of life.