Squamous Cell Carcinoma Developing in Epidermolysis Bullosa Dystrophica

Abstract

Two patients with epidermolysis bullosa dystrophica recessiva who had squamous cell carcinoma are presented. Case 1 is a 40-year-old woman who had ulcers on her left lower leg. Case 2 is a 42-year-old man who had a tumor on his left first toe. Wide surgical excision with skin coverage by autograft was performed in case 1. Amputation of the toe in case 2 was performed. A review of the cases of epidermolysis bullosa dystrophica associated with cancer reported in Japan is also presented.

Congenital Cerebellar Malignant Rhabdoid Tumor in an Infant with Junctional Epidermolysis Bullosa

“…His death was caused by Pseudomonas sepsis. Neoplasms are an uncommon but well-recognized complication of EB, especially SCC, which nearly always arises from the skin, but there is 1 report of this type of tumor arising in a bronchus [4,[6][7][8][9][10][11][12]. In contrast to our patient, neoplasms complicating or in association with EB nearly always occur in older children or adults (Table 1).…”

Subungual Squamous Cell Carcinoma of the Toe: Report on Three Cases

“…The cause of primary SCC of the nail bed is unknown. Factors considered to play a pathogenetic role include trauma, 14 chronic bacterial (osteomyelitis) 5,13 or viral (treatment‐resistant periungual or subungual warts) 12,23,24 infection, immune suppression, 12,23 tar, arsenic 25 or radiation exposure, 9,14 and inherited dermatologic disorders, including ectodermal dysplasia, 22 dyskeratosis congenita, 26 and dystrophic epidermolysis bullosa 6 . A case developing after a glomus tumor of the nail bed has also been described 4…”

“…PRIMARY TUMORS of the nail bed may be either in situ 1 or invasive 2–16 squamous cell carcinomas (SCC) or malignant melanomas 17 . Verrucous carcinomas 18,19 and keratoacanthomas 20 have also been described.…”

Management of cutaneous squamous cell carcinoma in patients with epidermolysis bullosa: best clinical practice guidelines

“…Different approaches to wound closure following excision of EB SCCs have been employed, including healing by secondary intention, 13,24,34,35,60,75 autologous split skin, [3][4][5]9,[13][14][15][16][18][19][20][21][22]24,33,39,41,43,44,48,49,53,55,57,59,73 epidermal 39 or full-thickness grafting, 2,32,40,48,50,58 allogeneic skin grafting, 28,38,58 cadaveric skin grafting, 58 artificial skin equivalents, 28,57,59 flaps, 28,50,[73][74][75] application of autologous or allogeneic keratinocyte suspension...…”