Опыт лечения плоскоклеточного рака кожи у пациентов с дистрофической формой буллезного эпидермолиза

Abstract

Epidermolysis bullosa (EB) is a hereditary, heterogeneous group of rare genetic dermatoses characterized by the skin and mucous membranes fragility. One of the most severe forms of the disease is dystrophic EB, the severity of which is due to the generalized nature of the lesion of the skin and mucous membranes of internal organs. In the absence of pathogenetic treatment of EB, multidisciplinary care is aimed at minimizing the risk of progression of skin lesions, relieving symptoms and specific complications. One of the most dangerous complications of EB is the early development of squamous cell skin cancer (SCC), which is especially characteristic for patients with dystrophic form. There are no generally accepted recommendations for the systemic therapy of SCC in EB, as well as clear evidence of the cytotoxic drugs clinical effectiveness in the treatment of this group of patients. The presented in the article experience of treatment of both localized and widespread forms of SCC in patients with dystrophic form of EB shows the possibilities of the surgical approach and the systemic therapy effectiveness. Of course, any experience in the treatment of a malignant tumor with such a severe orphan disease is of great value and can help in the selection of the most effective treatment tactics in the future.