title: Hailey-Hailey disease: A fold (intertriginous) dermatosis.
abstract: Hailey-Hailey disease, also called benign familial pemphigus, is a late-onset blistering disorder that affects the flexures. There are typically painful erosions and cracks in affected areas. Lesions generally begin between 20 and 40 years of age. In two third of all cases, positive family history is detected. In pathogenesis, there is a defect in keratinocyte adhesion due to ATP2 C1 gene mutation. The result of the desmosomal decomposition is acantholysis. Menstruation, pregnancy, skin infections, physical trauma, excessive sweating and exposure to ultraviolet radiation are important triggering factors. Histopathologic changes are suprabasal acantholysis and formation of intraepidermal bullae. In the epidermis, a partial acantholysis that looks like broken bricks is observed.
authors.affiliation: Department of Dermatology, Cerrahpaşa Medical Faculty, Istanbul University, Fatih, Istanbul 34098, Turkey. Electronic address: Burhanengin2000@yahoo.com.
authors.forename: Burhan
authors.initials: B
authors.lastname: Engin
authors.affiliation: Department of Dermatology, Cerrahpaşa Medical Faculty, Istanbul University, Fatih, Istanbul 34098, Turkey.
authors.forename: Zekayi
authors.initials: Z
authors.lastname: Kutlubay
authors.affiliation: Department of Dermatology, Cerrahpaşa Medical Faculty, Istanbul University, Fatih, Istanbul 34098, Turkey.
authors.forename: Uğur
authors.initials: U
authors.lastname: Çelik
authors.affiliation: Department of Dermatology, Cerrahpaşa Medical Faculty, Istanbul University, Fatih, Istanbul 34098, Turkey.
authors.forename: Server
authors.initials: S
authors.lastname: Serdaroğlu
authors.affiliation: Department of Dermatology, Cerrahpaşa Medical Faculty, Istanbul University, Fatih, Istanbul 34098, Turkey.
authors.forename: Yalçın
authors.initials: Y
authors.lastname: Tüzün
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D017677:Age Distribution; D017668:Age of Onset; D000890:Anti-Infective Agents / Q000627:therapeutic use; D001707:Biopsy, Needle; D017679:Cryotherapy / Q000379:methods; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D015994:Incidence; D007402:Intertrigo / Q000453:epidemiology* / Q000473:pathology* / Q000628:therapy; D053685:Laser Therapy / Q000379:methods; D008297:Male; D016506:Pemphigus, Benign Familial / Q000453:epidemiology* / Q000473:pathology* / Q000628:therapy; D011247:Pregnancy; D012008:Recurrence; D018570:Risk Assessment; D012720:Severity of Illness Index; D017678:Sex Distribution; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones; D000890:Anti-Infective Agents