J Korean Soc Microsurg.  2010 Nov;19(2):97-100.

Pedicled Deep Inferior Epigastric Perforator Flap for Treatment of Dystrophic Epidermolysis Bullosa-Associated Squamous Cell Carcinoma in the Groin: Case Report

• Kim KP
• Kim JH
• Kim ES
• Hwang JH
• Kim KS
• Lee SY

Abstract

PURPOSE
Epidermolysis bullosa is a rare genetic disease, characterized by the presence of extremely fragile skin and formation of recurrent blister resulting from even a minor mechanical injury. Squamous cell carcinoma (SCC) is recognized as a complication of the chronic scarring associated with dystrophic epidermolysis bullosa (DEB). When a soft tissue defect happens in a patient with epidermolysis bullosa, it is difficult to cover it with a skin graft or a flap. We describe the successful use of a pedicled deep inferior epigastric perforator flap for the reconstruction of SCC associated with DEB in the groin.
METHODS
A 29-year-old man diagnosed with DEB at birth sustained an ulcer increasing in the right groin for the last 7 months. Under general anesthesia, the mass lesion and lymph nodes were removed and the resulting defect was covered with a pedicled deep inferior epigastric perforator flap.
RESULTS
The flap survived completely and his postoperative course was uneventful. Histopathological examination revealed a SCC in the right groin and malignant tumor cells in the removed lymph nodes as well. Additional positron emission tomogram showed a malignant lesion in the ileocecal area with regional lymph node metastasis. The patient was referred to an oncologist for chemotheraphy, but the patient refused to take it. During a 4-month follow-up period, there was no recurrence in the right groin.
CONCLUSION
We suggest that perforator flaps can be considered as a reliable alternative for the reconstruction of soft tissue defects in a patient with DEB.

Keyword

Dystrophic epidermolysis bullosa; Squamous cell carcinoma; Perforator flap

MeSH Terms

Adult
Anesthesia, General
Blister
Carcinoma, Squamous Cell
Cicatrix
Electrons
Epidermolysis Bullosa
Epidermolysis Bullosa Dystrophica
Follow-Up Studies
Groin
Humans
Lymph Nodes
Neoplasm Metastasis
Parturition
Perforator Flap
Recurrence
Skin
Transplants
Ulcer