engleski

Squamous cell carcinoma in patients with epidermolysis bullosa: report of three cases

Cutaneous squamous cell carcinoma (SCC) is the most significant and serious complication in patients with inherited epidermolysis bullosa (EB). We present three patients with recessive dystrophic severe generalized EB (previously called Hallopeau Siemens) with cutaneous squamous cell carcinoma. In all three patients the tumours developed on distal parts of the limbs, that were exposed to chronic nonhealing erosions and ulcerations, which were finally severely mutilated. One patient had two primarily sites of SCC, the dorsum of the foot and the sacral region. No regional or distant metastases were detected. All patients had surgical treatment ; in two patients partial limb amputations and in one patient excision of the tumour with wide surgical margins were performed. Cutaneous squamous cell carcinoma is predominantly seen in patients with dystrophic epidermolysis bullosa (DEB). The prevalence of SCC in recessive DEB Hallopeau Siemens is 23% with the cumulative risk of 90% by 55 years of age. These tumours have aggressive and fast grow and metastasise early representing the leading cause of death in these patients. Multiple appearance of SCC in EB patients is often observed. Early age of development is a rule and it has been described as early as 14 years of age. Several biopsies may be required to confirm or exclude the presence of tumour within a suspicious area. Surgical treatment is the treatment of choice for SCC with amputations that are often indicated. Regular follow-up of non-healing wounds with early recognition of SCC is mandatory for effective treatment in patients with epidermolysis bullosa.

squmous cell carcinoma; epidermolysis bullosa; treatment

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