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Case Reports
. 2021 Feb 23;21(1):100.
doi: 10.1186/s12893-021-01105-6.

Dermatofibrosarcoma protuberans in a young patient with epidermolysis bullosa: a case report

B Bonaventura  1 , D Kraus  2 , G B Stark  2 , H Fuellgraf  3 , J Kiefer  2
Affiliations
Case Reports

Dermatofibrosarcoma protuberans in a young patient with epidermolysis bullosa: a case report

B Bonaventura et al. BMC Surg. .

Abstract

Background: Epidermolysis bullosa is a group of rare inherited skin diseases characterized by blister formation following mechanical skin trauma. Epidermolysis bullosa is associated with increased skin cancer rates, predominantly squamous cell carcinomas, yet to our best knowledge, there is no reported case of dermatofibrosarcoma protuberans in a patient with Epidermolysis bullosa.

Case presentation: Here, we present a 26-year-old man with junctional epidermolysis bullosa, who developed a DFSP on the neck. Initial, the skin alteration was mistakenly not considered malignant, which resulted in inadequate safety margins. The complete resection required a local flap to close the defect, which is not unproblematic because of the chronic inflammation and impaired healing potential of the skin due to Epidermolysis bullosa.

Conclusions: To our best knowledge, this is the first reported case of a skin-associated sarcoma in a patient with EB; however, further investigation is required to verify a correlation.

Keywords: Case report; Dermatofibrosarcoma protuberans; Epidermolysis bullosa; Local skin flap; Reconstructive surgery.

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Conflict of interest statement

The authors declare they have no competing interests.

Figures

Fig. 1
Fig. 1
Preoperative marking of the estimated skin resection ensures adequate safety margins, including the scar from the initial biopsy (incomplete resection). Orientation markings are essential for a complete histological workup
Fig. 2
Fig. 2
Histopathological image of the dermal DFSP infiltration. Hematoxylin and Eosin (HE) staining shows a dermal infiltration of a basophilic proliferation (green arrow) in accordance with residues of DFSP (a). The spindle cell proliferation with minimal atypia, a whorling growth pattern (b), and positivity for CD34 (c)
Fig. 3
Fig. 3
Local flap design for soft tissue coverage. Schematic image of the rhomboid flap (Limberg-Flap) to cover the wound defect
Fig. 4
Fig. 4
Clinical follow-up 10 months postoperatively. Clinical assessment of the neck with resulting hypertrophic scars ten months postoperatively. The markings indicate the original scar. A prolonged postoperative wound healing and muscle tension led to the visible scar alteration
See this image and copyright information in PMC

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