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...

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Publicado en:Dermatological Nursing Vol. 19; no. 4; pp. 10 - 17
Autores principales: Downe, Annette, Clapham, Jane
Formato: case study pictorial review tables/charts Journal Article
Publicado: British Dermatological Nursing Group Dec2020
Acceso en línea:Ver este registro en EBSCOhost
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      dt: Dec2020
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      pub: British Dermatological Nursing Group
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        atl: SQUAMOUS CELL CARCINOMA IN EPIDERMOLYSIS BULLOSA.
      aug:
        au:
          Downe, Annette
          Clapham, Jane
        affil: EB CNS at St John's Institute of Dermatology Guy's and St Thomas NHS Foundation Trust
      sug:
        subj:
          Carcinoma, Squamous Cell Diagnosis
          Epidermolysis Bullosa Classification
          Skin Diseases, Genetic
          Practice Guidelines
          Dermatology Nursing
          Epidermolysis Bullosa Familial and Genetic
          Carcinoma, Squamous Cell Therapy
          Carcinoma, Squamous Cell Risk Factors
          Collagen
          Early Diagnosis
          Male
          Aged
          Lymph Nodes
          Neoplasm Metastasis
          Epidermal Growth Factors
          Chemotherapy, Cancer Methods
          Female
          Adult
          Amputation
          Rare Diseases
          Aged: 65+ years
          Adult: 19-44 years
          Male
          Female
      ab: 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.
      pubtype: Academic Journal
      doctype:
        case study
        pictorial
        review
        tables/charts
        Journal Article
      ougenre: Article
    language: English
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