Dermatol. praxi. 2024;18(1):39-43 | DOI: 10.36290/der.2024.006

Morbus Hailey-Hailey

MUDr. Eliška Langerová

Dermatologická ambulance, Olomouc

Morbus Hailey-Hailey, benign familiar pemfigus, is a chronic autosomal dominant acantholytic dermatosis, which is characterised by flaccid bullae in the intergtriginous areas that rupture easily. In chronic lesions appear erythematous plaques, moist vege­tations, painful fissures and lesions are malodorous. Disease has relapsing-remitting course, with worsening during the summer. Trigger factors are UV light, friction, sweating, infection, stress, hormonal changes. It affects mainly people in their 30´s-40´s, without sex or racial predominance. Positive family history is in 70 % of cases. Defect in ATP2C1 gene, localised at 3^rd chromosome is responsible for dysregulation of Ca²⁺ transport in cells, which influences keratinocytic adhesion, followed by acantholysis throughout the whole epidermis with formation of suprabasal clefting and with a typical histological picture of dilapitated brick wall. For treatment we use topical corticosterids, antibiotics, antimycotics, combined topical treatment, local imunomodulators. Systemically we use corticosteroids, antibiotics, antimycotics, antivirotics and disease-modifying antirheumatic drugs. Patients can as well undergo laser therapy, phototherapy, botulotoxin A injections, dermabrasion and other surgical methods. In a following case report we present 2 cases of patients with Hailey-Hailey disease treated in our dermatological office.

Keywords: Morbus Hailey-Hailey, genodermatosis, ATP2C1, acantholysis, relapsing-remiting course.

Accepted: March 26, 2024; Published: April 3, 2024  Show citation