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We report a patient with naevoid basal cell carcinoma syndrome (NBCCS) who developed cutaneous cysts on her digits. Histological examination of one of the cysts showed a festooned lining epithelium maturing without a granular cell layer, similar to that of the jaw keratocyst characteristic of this syndrome. This type of cutaneous keratocyst has been reported only once previously. in two patients with NBCCS.
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