Cutaneous Squamous Cell Carcinoma in Epidermolysis Bullosa: a 28-year Retrospective Study

Authors

  • Susan J. Robertson
  • Elizabeth Orrin
  • Manpreet K. Lakhan
  • Gavin O'Sullivan
  • Jessie Felton
  • Alistair Robson
  • Danielle T. Greenblatt
  • Catina Bernardis
  • John A. McGrath
  • Anna E. Martinéz
  • Jemima E. Mellerio St John's Institute of Dermatology, Guy's and St Thomas' NHS Foundation Trust, London SE1 7EH, United Kingdom

DOI:

https://doi.org/10.2340/00015555-3875

Keywords:

epidermolysis bullosa, squamous cell carcinoma, cancer, prognosis

Abstract

Epidermolysis bullosa (EB), notably severe recessive dystrophic EB (RDEB-S), is associated with increased risk of aggressive mucocutaneous squamous cell carcinomas, the major cause of mortality in early adulthood. This observational, retrospective case review describes a series of EB patients with cutaneous squamous cell carcinomas over a 28-year period. Forty-four EB patients with squamous cell carcinomas were identified with a total of 221 primary tumours. They comprised: 31 (70%) with RDEB-S, 4 (9%) with other RDEB subtypes, 5 (11.4%) with dominant dystrophic EB, 3 (6.8%) with intermediate junctional EB and 1 (2.3%) with Kindler EB. Squamous cell carcinomas occurred earlier in RDEB-S (median age 29.5 years; age range 13–52 years) than other groups collectively (median age 47.1 years; age range 30–89 years) and most had multiple tumours (mean 5.8; range 1–44). Squamous cell carcinoma-associated mortality was high in RDEB-S (64.5%), with median survival after first squamous cell carcinoma of 2.4 years (range 0.5–12.6 years), significantly lower than previous reports, highlighting the need for early surveillance and better treatments.

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References

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Published

2021-08-24

How to Cite

Robertson, . S. J., Orrin, . E., Lakhan, . M. K., O’Sullivan, . G., Felton, . J., Robson, . A., Greenblatt, . D. T., Bernardis, . C., McGrath, J. A., Martinéz, A. E., & Mellerio, J. E. (2021). Cutaneous Squamous Cell Carcinoma in Epidermolysis Bullosa: a 28-year Retrospective Study. Acta Dermato-Venereologica, 101(8), adv00523. https://doi.org/10.2340/00015555-3875