Clinical Cases of Darier-White Follicular Dyskeratosis
October 2022
in “
Medičnì perspektivi
”
TLDR Darier-White disease causes skin and nail issues, starts around age 20, worsens until 40-50, and has poor treatment options.
Darier-White disease, a rare hereditary skin disorder caused by a mutation in the ATP2A2 gene, presents with follicular papules and nail abnormalities, affecting both genders equally but more severely in males. It typically manifests around age 20 and progresses until 40-50, then gradually diminishes. Clinical features include yellow-brown papules that merge into plaques, primarily on the limbs, trunk, and large folds, with 20-30% of cases also affecting the oral mucosa. Dermatoscopy reveals distinctive yellow-orange-brown lumps. The prognosis is poor, and treatment is largely ineffective. The article details two clinical cases of this disease.
