Papular acantholytic dermatosis of the vulva is a rare,chronic disorder and is an entity that remains to be fullyunderstood. It shares clinical and histopathologicaloverlap with Darier disease and Hailey-Hailey disease.We describe a 30-year-old woman with papularacantholytic dermatosis of the vulva. The lesionsconsisted of whitish papules and erosions on the labiamajora. Histologically, there was hyperkeratosis andfocal parakeratosis with acantholytic and dyskeratoticcells. She did not respond completely to topicalsteroids but clinical improvement occurred after theuse topical tacrolimus
