SQUAMOUS CELL CARCINOMA IN EPIDERMOLYSIS BULLOSA.
Epidermolysis bullosa (EB) refers to a group of rare genetically inherited skin fragility disorders where the skin and mucous membrane blister or shear in response to minimal friction or trauma. The four main types range in severity and its impact on an individual's quality of life. The shared commo...
| Publicado en: | Dermatological Nursing Vol. 19; no. 4; pp. 10 - 17 |
|---|---|
| Autores principales: | , |
| Formato: | case study pictorial review tables/charts Journal Article |
| Publicado: |
British Dermatological Nursing Group
Dec2020
|
| Acceso en línea: | Ver este registro en EBSCOhost |
| Sumario: | Epidermolysis bullosa (EB) refers to a group of rare genetically inherited skin fragility disorders where the skin and mucous membrane blister or shear in response to minimal friction or trauma. The four main types range in severity and its impact on an individual's quality of life. The shared commonality in all types of EB are life-long wounding with cutaneous and systemic complications. However, people with more severe forms of EB are at increased risk of developing aggressive squamous cell carcinoma (SCC) often leading to an early death. Unlike in the general population, these tumours tend to develop rapidly and metastasise. This article will focus on the difficulty of diagnosis due to the broad clinical appearance of tumours and underlines the importance of a multidisciplinary patient centred approach which focuses on early detection and treatment of these aggressive SCCs. Advances in targeted therapies has the potential of changing treatment, but the current gold standard treatment remains surgical excision. The case studies demonstrate the vital role that the expert patient plays in their care which is key to optimising the best outcome. |
|---|