Vulval squamous cell carcinoma arising in localized Darier’s disease

Darier’s disease (keratosis follicularis) is a rare vulval lesion and it has been associated with squamous and non-squamous tumors from different origin. A vulval squamous cell carcinoma (SCC) arising in a localized Darier’s disease is presented. To our knowledge, this is the first case to report the association of a vulval SCC and a localized Darier’s disease. The possibility of delaying or overlooking the diagnosis of a vulval carcinoma is also illustrated. It is suggested to resect the whole lesion of Darier’s disease localized to the vulva or take multiple biopsies.