Darier disease (DD) also known as keratosis follicularis is a rare autosomal dominant (AD) inheritance disease, with reportedly approximately 40% sporadic cases. Manifestations usually begin at childhood and persist through adulthood. DD is characterised by loss of intercellular adhesion and disordered keratinisation with macroscopic picture of greasy papules and plaques in the seborrheic areas. There are currently no validated curative treatments available. The goal is to present a severe type of generalised DD and our clinical observation and treatment over the years. A male patient with no family history of such disease developed DD as an infant with mild clinical manifestation, that become severe in adolescent and lives with generalised form for more than 50 years. He presented with generalised form of DD, livid disseminated hyperkeratotic papules and rhagades scattered throughout the whole body, with characteristic nail changes and facies leonine. Over the years he was frequently hospitalised for various secondary skin infections with sepsis, rhagadiforme inflammations, skin cracks and in forefront painful hypertrophic vegetation’s in skin-fold areas, with subcutaneous nodules and abscesses. Diagnosis was confirmed with histopathology skin biopsy due to presentation of dyskeratosis and acantholysis leading to supra basal cleavage. With DIF method primary blistering disease was excluded. Over the years we tried treatment therapy with oral retinoids, calcineurin inhibitors, systemic antibiotics and corticosteroids, topical therapies such as tretinoin, corticosteroids, antibiotics, anti-fungal agents, emollients and antiseptic solutions. Moreover, with phototherapy, for proliferative lesions electrocoagulation and surgical dissection. For some time, clinical picture improved, however the lesions relapsed. DD usually persist for life and is characterized by relapsing-remitting course. It is challenging to control and treat and can result in frequent hospitalisations due to complications. Quality of life is adversely impacted by malodour and psychosocial consequence of appearance. Understanding pathogenesis of DD has advanced greatly in recent years but there are still many questions to be answered.

Keywords: Darier diseases, Dyskeratosis follicularis, Genodermatosis