Cutaneous squamous cell carcinoma in epidermolysis bullosa: A 28-year retrospective study

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. Martinez, Jemima E. Mellerio*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

18 Citations (Scopus)

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.

Original languageEnglish
Article numberadv00523
JournalActa Dermato-Venereologica
Volume101
Issue number8
DOIs
Publication statusPublished - Aug 2021

Keywords

  • Cancer
  • Epidermolysis bullosa
  • Prognosis
  • Squamous cell carcinoma

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