Squamous cell carcinoma complicating recessive dystrophic epidermolysis bullosa-Hallopeau-Siemens: a report of four cases

Abstract

Regular clinical and histologic examination of any nodular lesions or non healing ulcers of all patients suffering from RDEB-Hallopeau-Siemens to detect an early malignancy is recommended.

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

“…However, the activities of DEBRA International will aid in dissemination of the guidelines and facilitate adoption by the proposed user groups. [11][12][13] Areas of skin clinically suspicious for SCC should be biopsied for histological evaluation D III-IV 12,14,17,22 Tumour evaluation and staging All patients with EB presenting with an SCC should have multidisciplinary review SCCs ≥ 5 cm diameter or overlying difficult anatomical sites should be imaged with magnetic resonance imaging or computed tomography to assess tumour extent D III 15,16,27 Lymphadenopathy should be assessed for potential metastatic SCC D III 2,4,9,13,[15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30][31][32][33] Patients with EB diagnosed with an SCC may require staging D III 4,13,20,22,24,27,34,35 Surgical treatment Wide local excision is the treatment of choice for EB SCCs D III [2][3][4]…”

“…2 If lymph node biopsy is positive for metastatic SCC, regional lymph node dissection should be considered. 2,9,13,15,19,21,[25][26][27][28][29][30] This can usually be carried out at the time of surgical excision of the primary SCC. 3 Sentinel lymph node biopsy (SLNB) may be undertaken in patients with EB presenting with SCC.…”

“…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...…”

“…SCCs may arise at a young age in patients with EB, particularly those with RDEB‐SG. This complication has been described in a child as young as 6 years of age, and has been reported frequently in patients aged < 20 years . As the incidence of SCC increases with age in at‐risk forms of EB, and patients frequently develop multiple primary tumours, ongoing monitoring for patients with previous SCCs becomes even more important (Table ).…”

Cytoskeletal protein flightless I inhibits apoptosis, enhances tumor cell invasion and promotes cutaneous squamous cell carcinoma progression

“…Cutaneous squamous cell carcinomas (SCCs) are invasive, recurrent and in some cases potentially metastatic [ 1 , 2 ]. Identifying new target proteins that inhibit cancer cell invasion and stimulate tumor apoptosis could lead to new treatments to reduce the severity of SCCs.…”