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

doi:10.2340/00015555-3875

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

Dermatology

Research output: Contribution to journal › Article › peer-review

6 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 language	English
Article number	adv00523
Journal	Acta Dermato-Venereologica
Volume	101
Issue number	8
DOIs	https://doi.org/10.2340/00015555-3875
Publication status	Published - Aug 2021

Keywords

Cancer
Epidermolysis bullosa
Prognosis
Squamous cell carcinoma

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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10.2340/00015555-3875

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title = "Cutaneous squamous cell carcinoma in epidermolysis bullosa: A 28-year retrospective study",

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.",

keywords = "Cancer, Epidermolysis bullosa, Prognosis, Squamous cell carcinoma",

author = "Robertson, {Susan J.} and Elizabeth Orrin and Lakhan, {Manpreet K.} and Gavin O{\textquoteright}sullivan and Jessie Felton and Alistair Robson and Greenblatt, {Danielle T.} and Catina Bernardis and McGrath, {John A.} and Martinez, {Anna E.} and Mellerio, {Jemima E.}",

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T2 - A 28-year retrospective study

AU - Robertson, Susan J.

AU - Orrin, Elizabeth

AU - Lakhan, Manpreet K.

AU - O’sullivan, Gavin

AU - Felton, Jessie

AU - Robson, Alistair

AU - Greenblatt, Danielle T.

AU - Bernardis, Catina

AU - McGrath, John A.

AU - Martinez, Anna E.

AU - Mellerio, Jemima E.

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Y1 - 2021/8

N2 - 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.

AB - 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.

KW - Cancer

KW - Epidermolysis bullosa

KW - Prognosis

KW - Squamous cell carcinoma

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ER -

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

Abstract

Keywords

UN SDGs

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