Abstract Number: 77

Unilateral segmental Darier’s disease: a case series

S.C. Cai, R.K. Chan, H.H. Oon

Meeting: 2021 Dermcoll

Session Information

Date: -

Session Title: Poster Presentations

Session Time: -

Darier’s disease is a rare autosomal dominant genoder- matosis characterized by multiple keratotic papules involv- ing mainly seborrheic areas. Only 10% feature a segmental localized pattern, a rare variant due to Type I or II mosaicism. We describe three cases of segmental Darier’s disease with histopathological confirmation. The first case was a 56-year-old female with brownish-red ker- atotic papules on the left neck and chest for one month, who responded to topical mometasone, tretinoin and cal- cipotriol cream. The second case was a 48-year-old male with linear brown papules on the right groin and scrotum for several months, who responded to carbon dioxide laser after failing topical steroid and calcineurin inhibitors. The third patient was a 70-year-old male who had a cluster of keratotic papules involving the left abdomen in a zosteri- form pattern for four months. Improvement in the lesions was seen with topical calcipotriol and clobetasol cream. There was no family history or any oral, nail or palmo- plantar involvement in these three cases. Their skin biop- sies shared similar findings of Darier-like acantholytic dyskeratosis with “corps ronds and grains” appearance. A diagnosis of segmental Darier’s disease was made. Differ- ential diagnoses include diseases that follow Blaschko’s lines such as inflammatory linear verrucous epidermal nevus, lichen striatus and linear lichen planus. Although features of later-onset, unilateral brown-red keratotic papules, seborrheic or flexural location may favour seg- mental Darier’s disease, histopathology is necessary to establish the diagnosis. Topical treatment may be used for mild disease with destructive surgical or laser therapy an option for localized recalcitrant lesions.