Management of squamous cell carcinoma in a patient with recessive-type epidermolysis bullosa dystrophica.
BACKGROUND
Surgical treatment of a recessive dystrophic epidermolysis bullosa patient is very difficult for both the surgeon and the anesthetist because of the fragility of the skin and abnormal nature of the tumor bed.


OBJECTIVE
We report a case of 54-year-old Japanese recessive dystrophic epidermolysis bullosa patient with squamous cell carcinoma (SCC) of the lateral malleolus.


METHODS
A tumor measuring 5.0 x 5.5 cm was surgically excised. The defect was then reconstructed by full-thickness skin grafting. To avoid airway complications, general anesthesia was administered using a face mask. Because the regional lymph nodes were swollen before surgery, the patient underwent sentinel lymph node biopsy.


RESULTS
The patient remains well with no sign of recurrence or metastasis 7 months after surgery.


CONCLUSIONS
To preserve activities of daily living, surgery should be performed for squamous cell carcinomas arising in recessive dystrophic epidermolysis bullosa patients.
Yamada
Mizuki
M
Department of Dermatology, Kanazawa University Graduate School of Medical Science, Kanazawa, Japan.
Hatta
Naohito
N
Sogo
Kana
K
Komura
Kazuhiro
K
Hamaguchi
Yasuhito
Y
Takehara
Kazuhiko
K
D002294:Carcinoma, Squamous Cell / Q000209:etiology* / Q000473:pathology / Q000601:surgery; D016108:Epidermolysis Bullosa Dystrophica / Q000150:complications* / Q000235:genetics / Q000473:pathology; D005808:Genes, Recessive; D006801:Humans; D008297:Male; D008875:Middle Aged; D012878:Skin Neoplasms / Q000209:etiology* / Q000473:pathology / Q000601:surgery