Table 1.

EB patients treated with cetuximab in the literature

	Age at tumor’s presentation (years)	Tumor characteristics	Lymph node metastases present	Organ metastases present	Treatment options before cetuximaba	Patient outcome under cetuximab	Reference
1	29	primary tumor on right forearm; axillary lymph node metastases as well as in-transit cutaneous metastases on the right upper limb	axillary	–	–	treatment initially well-tolerated, but wound healing deficits after 6 months; progression free survival for 9 months; treatment discontinuation after 2 years after grade 2 allergic reaction; death 1 year later after use of other treatment options	Medek et al., J Dtsch Dermatol Ges. 2019
2	24	primary well-differentiated SCC on right elbow; subcutaneous metastasis next to SCC; tumor regrowth within weeks after 3x excisions (amputation declined by patient)	2 axillary	–	radiotherapy; chemotherapy with cisplatin and 5-fluorouracil (2 cycles)> > chemotherapy with cisplatin and paclitaxel for 1 month> > cetuximab for 12 cycles	besides acneiform folliculitis on the face treatment well-tolerated; progression free survival for 3 months	Arnold et al., Dermatology, 2009
3	26	moderately differentiated SCC on the dorsal right hand; quick tumor regrowth after excision, resulting in amputation	2/22 axillary	lung	axillary dis-section followed by local radiotherapy> > cetuximab for 10 cycles> > due to progression of lung metastasis combination with gemcitabine	treatment well tolerated; death around 3 weeks after initiation of cetuximab+ gemcitabine treatment on pneumonia	Kim et al., Br J Dermatol. 2013
4	43	well-differentiated SCC in axilla with extensive extranodal local spread; recurrence in axilla within 3 months after axillary node dissection	6/16 axillary	lung	axillary dis-section followed by local radiotherapy; cetuximab for 7 cycles> > due to development of multiple lung metastases: methotrexate 40 mg orally and then i.v. for 9 weeks> > treatment cessation due to progressive disease	initially development of a vesicular eruption, no other side effects; death 2 months after treatment discontinuation on pneumonia	Kim et al., Br J Dermatol. 2013
5	na	na	na	na	na	worsening of skin lesions	Maubec et al., J Clin Oncol. 2011

All patients published so far had severe generalized recessive DEB

Legend: +, present; −, absent; ^a, treatment options are presented in chronological order; na not available