Hypopigmented Segmental Darier Disease

Abstract

To our knowledge, this is the first case reporting a combined segmental and hypopigmented variant of Darier disease. We further present a literature review for hypopigmented and segmental variants of Darier disease.

Hypopigmented macules and papules following the lines of Blaschko: a novel variant of Darier's disease

“…Of note, the mosaic variant may manifest as unilateral streaks of keratotic papules (type 1) or with increased severity of similar lesions in a streaky pattern over a background of generalized disease (type 2) . Morin et al . recently reported a 41‐year‐old, African‐Canadian woman with hypopigmented macules and papules that followed Blaschko's lines on the scalp, trunk, and upper extremities, and showed histological features of Darier's disease.…”

Guttate leukoderma in darier disease: A rare presentation

“…Several variants of DD have been reported, including the guttate hypopigmented, vesiculobullous, acral hemorrhagic, acneiform comedonal, cornifying, and segmental or linear variants. [2] Small leukodermic macules were first described by Goodall and Richmond in a patient of DD in 1965. [3] Hypopigmented macules in DD is rare.…”

“…Some authors considered guttate leukoderma as postinflammatory or subclinical form of DD; however, the increasing number of reported cases devoid of papular lesions or macules evolving into hyperkeratotic papules refute the above hypothesis. [2] Another proposed hypothesis is dysregulated melanin transfer due to lack of keratinocyte adhesion caused by the ATP2A2 gene mutation leading to guttate leukoderma. [5]…”

“…Histological examinations of hypopigmented DD variant vary from barely seeing any typical disease features to classic dyskeratosis with the presence of corps ronds and grains and acantholysis. [2]…”

“…Treatment modalities include emollients, topical steroids and calcineurin inhibitors, topical 5-fluorouracil, oral retinoids, laser treatments, and surgery. [2] Increasing awareness regarding guttate leukodermic variant of DD is important to prevent misdiagnosis and for appropriate management.…”