Congenital acantholytic dyskeratotic dermatosis: localized Darier disease or disseminated benign papular acantholytic dermatosis?

Acantholytic dyskeratosis is a histopathologic pattern defined by a hyperkeratotic and parakeratotic epidermis with intraepidermal clefts containing acantholytic and dyskeratotic keratinocytes. These typical features are distinctive but not entirely pathognomonic of Darier disease, since they may occur at cutaneous and mucocutaneous sites in other conditions such as Grover disease, acantholytic and dyskeratotic epidermal nevus (nevus of Starink), warty dyskeratoma, or acantholytic papular dermatosis localized to the vulvocrural area. We report a newborn girl who had congenital erosive papules and plaques located on the left thigh, left ankle, and right side of the neck. Histopathologic examination of a punch biopsy specimen disclosed findings typical of acantholytic dyskeratosis. In the absence of any family history or other manifestations of Darier disease, we propose the descriptive term "congenital acantholytic dyskeratotic dermatosis." This descriptor characterizes our patient's disease on the basis of the clinical and histopathologic findings and facilitates recognition of this condition until a putative genetic mutation can be demonstrated or ruled out.