Management of squamous cell carcinoma in a patient with dominant-type epidermolysis bullosa dystrophica: a surgical challenge.
Epidermolysis bullosa dystrophica is a rare congenital skin disease inherited either as a recessive or dominant form, the latter form being less common and much less severe. Squamous cell carcinoma is a rare complication in the dominant form of the disease, only three such cases being reported before, making this case the fourth known case. Although the squamous cell carcinoma is well-differentiated by histopathology, it has a poor prognosis owing to its invasiveness, distant metastases, and multicentricity. Its management also poses a great challenge to the surgeon and personnel involved in patient care. A complete outline of the total management of such a case is described with some interesting observations not mentioned previously in the literature. All the previous three patients are deceased, and this is the only known surviving patient.
D000328:Adult; D002294:Carcinoma, Squamous Cell / Q000150:complications* / Q000473:pathology / Q000601:surgery; D004820:Epidermolysis Bullosa / Q000150:complications* / Q000473:pathology / Q000601:surgery; D006801:Humans; D007430:Intraoperative Care; D008297:Male; D011182:Postoperative Care; D012878:Skin Neoplasms / Q000150:complications* / Q000473:pathology / Q000601:surgery