Inherited epidermolysis bullosa and squamous cell carcinoma: a systematic review of 117 cases

Table 5 Systemic treatment of cSCC in EB patients

Reference (s)	Age (yr)/sex	EB subtype	Site of cSCC	Histological differentiation/size of tumor (cm)	Site(s) of metastases	Treatment	Outcome
Schwartz¹⁶	55 F	DDEB	Lower extremity (knee)	Well/>5	Not applicable	Intra arterial Doxorubicin + MTX	Surgical excision Survival unknown
Lentz¹⁷	22 F 26 F	RDEB	Upper extremity (forearm) Trunk	Well/unknown Unknown/>2 < 5	Axillary lymph nodes, pulmonary nodules Axillary lymph nodes	Cisplatin 5FU-cisplatin	PR Death from cSCC PR, surgical resection, alive at 12mo
Arnold⁷	24 F	RDEB	Upper (elbow) and lower (feet) extremities	Well/>5	Axillary lymph nodes	1.5FU-cisplatin 2. carboplatin-taxol 3. cetuximab	1. PR 2. PD 3. GR, alive at 3mo
Kim¹⁸	26 F 43 M	RDEB	Upper (hand) extremity Unknown	Moderately/>5 Well/>5	Axillary lymph nodes, pulmonary nodules Axillary lymph nodes, pulmonary nodules	1. cetuximab 2. cetuximab-gemcitabine 1. cetuximab 2. MTX orally 3. MTX IV	1. PD 2. Death from pneumonia 1. PD 2. Death from pneumonia

cSCC squamous cell carcinoma, cm centimeter, DDEB dominant dystrophic epidermolysis bullosa, F female, GR good response, EB inherited epidermolysis bullosa, IV intravenously, M male, mo month, MTX methotrexate, PD progressive disease, PR partial response, RDEB recessive dystrophic epidermolysis bullosa, Yr year, 5FU 5 fluorouracil