Summary

Darier disease is a rare autosomal dominant genodermatosis with onset in the first two decades of life. The classical form of Darier’s disease is described in the literature as a persistent eruption of keratotic, hyperpigmented or red-brown papules with distribution in seborrheic and intertriginous areas. Although the severity of the pathology is variable, it usually has a chronic course with frequent exacerbations. We report on the case of a 41-year-old patient with Darier’s disease with a typical pattern, moderate involvement and with a family medical history of Darier disease in a first-degree relative. Although clinically, the differential diagnosis can pose some problems, requiring the exclusion of other pathologies with a similar distribution, such as Hailey-Hailey disease or Grover’s disease, the morphopathological diagnosis is the one of certainty in this situation. We emphasize the therapeutic challenges, since Darier’s disease does not have a curative treatment, and remissions are frequent. The emphasis in this presentation is on the favorable therapeutic response, despite the low compliance, the anxious character and the psychosocial deterioration that impacted the patient’s life along with the evolution of the disease.