Debilitating Darier's disease

Darier's disease is an inherited dermatitis that is due to a mutation in the ATP2A2 gene. This mutation causes disrupted signaling within cells and leads to a skin condition that is resistant to many dermatologic treatments. Though serious complications are rare, Darier's disease can result in bacterial sepsis and Kaposi's varicelliform eruption. In some families seizure disorders are linked to Darier's disease. There is also a paraneoplastic variant that could be considered in patients without a family history of Darier's disease. This article reports a case with typical verrucous masses and waxy papules which, over twenty years, became disabling. Symptoms included pruritus and foul odor and were relieved, along with the keratotic rash, utilizing systemic treatment with acitretin though the condition recurred rapidly when treatment was interrupted. Darier's disease must be considered when patients present with hyperkeratotic lesions that are resistant to usual forms of therapy for inflammatory dermatoses.