Syn.: Segmental Hailey-Hailey disease, relapsing linear acantholytic dermatosis.
Def .: Epidermal nevus clinically and histopathologically corresponding to changes in Hailey-Hailey disease.
Epid .: It is rare.
Etiol .: A mosaic variety caused by a somatic mutation during embryogenesis.
Pat: The mutation concerns the ATP2C1 gene, which encodes calcium ion-dependent ATPase (sarcoplasmic / endoplasmic reticulum Ca2 + ATP-ase 2 – SERCA 2), an enzyme responsible for the formation of intercellular connections and cell adhesion.
Loc .: One-sided, along the nerve segments.
Clinical: Vesiculo-erosive foci may be accompanied by slightly intensified lesions with a symmetrical arrangement in the areas typical for Hailey-Hailey disease (neck, armpits, groin).
Hist .: Increased acantholysis beginning above the basal layer and covering the entire epidermis.
DR: Shingles, a mosaic form of the following diseases: Darier’s disease, Brocq epidermolytic hyperkeratosis, type I neurofibomatosis, multiple syringomas, diseminated superficial actinic porokeratosis.
Healing: Steroid ointments, tacrolimus ointment, surgical removal of treatment-resistant lesions.
Year .: Recurring mileage.
Lit.: [1] Bittar M., Happle R.: ‘Atypical’ cases of haileyhailey disease may represent typical examples of a type 2 segmental manifestation. Dermatology 2005, 210; 182-3. [2] Ohtsuka T., Okita H., Hama N., Yamazaki S.: Novel mutation in ATP2C1 gene in a Japanese patient with Hailey- Hailey disease. Dermatology 2006, 212; 194-7.
Source: A. Kaszuba, Z. Adamski: “Lexicon of dermatology”; XNUMXst edition, Czelej Publishing House