Syn.: Segmental Darier disease, acantholytic dyskeratotic epidermal nevus.
Def .: Epidermal nevus clinically and histopathologically (acantholysis and dyskeratosis) corresponding to changes in Darier’s disease.
Epid .: It accounts for about 10% of cases of Darier’s disease, which affects 1 in 55.
Etiol .: Mosaic form of Darier’s disease caused by somatic mutation during embryogenesis
Pat: The mutation concerns the ATP2A2 gene located in the 2-cM region of chromosome 12, 12q23-24, which encodes calcium ion-dependent ATPase (sarcoplasmic / endoplasmic reticulum Ca2 + ATP-ase 2 – SERCA2), an enzyme responsible for the formation of intercellular connections and cell adhesion.
Loc .: One-sided along the Blaschko line.
Clinical: Limited hyperkeratotic foci with a linear system, on the diseased side may be accompanied by changes in the hands and nails typical of Darier’s disease.
Hist .: Features of acantholysis and abnormal keratosis (dyskeratosis).
DR: Shingles, mosaic form of the following diseases: epidermolytic hyperkeratosis Brocq, neurophilic bomatosis type I, multiple syringomas, diseminated superfi cial actinic porokeratosis, Hailey-Hailey disease.
Healing: Retinoids topically (not very effective) or orally (side effects!).
Year: Recurrent course, exacerbation under the influence of sun, warming up, sweating, mechanical irritation
Lit.: [1] Gilaberte M., Puig L., Vidal D., Alomar A.: Acantholytic dyskeratotic naevi following Blaschko’s lines: a mosaic form of Darier’s disease. J Eur Acad Dermatol Venereol 2003, 17; 196-9. [2] Goldberg E.I., Lefk ovits A.M., Sapadin A.N.: Zosteriform Darier‘s disease versus acantholytic dyskeratotic epidermal nevus. Mt Sinai J Med 2001, 68; 339-41. [3] Huh W.K., Fujiwara K., Takahashi H., Kanitakis J.: Congenital acantholytic dyskeratotic epidermal naevus following Blaschko‘s lines versus segmental Darier‘s disease. Eur J Dermatol 2007, 17; 130-2.
Source: A. Kaszuba, Z. Adamski: “Lexicon of dermatology”; XNUMXst edition, Czelej Publishing House