TY - JOUR
T1 - Epidermolysis bullosa complicated by squamous cell carcinoma
T2 - report of 10 cases
AU - McGrath, J. A.
AU - Schofield, O. M.V.
AU - Mayou, B. J.
AU - McKee, P. H.
AU - Eady, R. A.J.
PY - 1992/4
Y1 - 1992/4
N2 - Epidermolysis bullosa (KB) refers to a group of hereditary mechano‐bullous conditions, many of which arc associated with chronic scarring. Several forms of (he disease have been reported in association with cutaneous malignancy. We present a series of 10 EB patients (eight generalised recessive dystrophic EB, one dominant dystrophic EB, one non‐lethal junctional EB) aged 24–25 years with a total of 29 squamous cell carcinomas (SCC). Three patients died from metastatic disease associated with invasive, poorly differentiated SCC. Six cases had multiple primary SCG, including three patients with simultaneous multifocal disease. Twenty‐eight of the 29 SCC arose on the limbs. I Histology revealed that most of the SCC were well or moderately differentiated (22/29). Unusual histological findings included two verrucous SCC, as well as a spindle cell (angiosarcoma‐like) SCC. Most of the SCC developed in areas of chronic non‐healing ulceration (10/29) or longstanding hyperkeratotic crusting (14/29). The dermis around or beneath the carcinomas was densely scarred, more so than in non‐malignant areas. In some cases it was difficult to distinguish the clinical appearances of certain areas of chronic ulceration, scarring, and crusting typical of dystrophic EB from many of the SCC. This study underlines the need for constant vigilance for the development of carcinomas in this group of patients, the occasional diagnostic difficulty, and the potential for metastasis.
AB - Epidermolysis bullosa (KB) refers to a group of hereditary mechano‐bullous conditions, many of which arc associated with chronic scarring. Several forms of (he disease have been reported in association with cutaneous malignancy. We present a series of 10 EB patients (eight generalised recessive dystrophic EB, one dominant dystrophic EB, one non‐lethal junctional EB) aged 24–25 years with a total of 29 squamous cell carcinomas (SCC). Three patients died from metastatic disease associated with invasive, poorly differentiated SCC. Six cases had multiple primary SCG, including three patients with simultaneous multifocal disease. Twenty‐eight of the 29 SCC arose on the limbs. I Histology revealed that most of the SCC were well or moderately differentiated (22/29). Unusual histological findings included two verrucous SCC, as well as a spindle cell (angiosarcoma‐like) SCC. Most of the SCC developed in areas of chronic non‐healing ulceration (10/29) or longstanding hyperkeratotic crusting (14/29). The dermis around or beneath the carcinomas was densely scarred, more so than in non‐malignant areas. In some cases it was difficult to distinguish the clinical appearances of certain areas of chronic ulceration, scarring, and crusting typical of dystrophic EB from many of the SCC. This study underlines the need for constant vigilance for the development of carcinomas in this group of patients, the occasional diagnostic difficulty, and the potential for metastasis.
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U2 - 10.1111/j.1600-0560.1992.tb01352.x
DO - 10.1111/j.1600-0560.1992.tb01352.x
M3 - Article
C2 - 1597567
AN - SCOPUS:0026596372
SN - 0303-6987
VL - 19
SP - 116
EP - 123
JO - Journal of Cutaneous Pathology
JF - Journal of Cutaneous Pathology
IS - 2
ER -