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Linear Darier disease

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Linear Darier disease
Mariame Meziane, Rim Chraibi, Nadia Kihel, Badreddine Hassam, Karima Senouci
Dermatology Online Journal 14 (12): 11

Department of Dermatology, Ibn Sina Hospital, Rabat, Morocco.


Darier disease is an uncommon genodermatosis characterized by verrucous papules in a seborrheic distribution. The linear form of this disease is rare and could result from genetic mosaicism in this autosomal dominant disorder. We report a case of linear Darier disease that involved the right lower limb with a zosteriform distribution.


Darier disease (DD), also known as Darier-White disease or keratosis follicularis, is an uncommon genodermatosis characterized by verrucous papules in a seborrheic distribution. Recent studies have characterized a somatic mutation in unilateral DD. We present a case of Darier disease that involved the right lower limb with a zosteriform distribution.

Figure 1Figure 2
Figure 1. Clinical photograph reveals hyperkeratotic vegetative and hyperpigmented papules with a zosteriform pattern.
Figure 2. Red longitudinal bands and subungual hyperkeratosis of nails

Case report

A 55-year-old man came to our department with a unilateral diffuse zosteriform eruption of 20-years duration. The eruption initially had appeared on the right side of his leg and over the course of months had spread distally to involve the entire lower leg with a zosteriform distribution. The eruption has remained stable over the past five years; because of its asymptomatic nature, the patient never sought medical attention. There was no history of a similar eruption in any other family members or in his three children. Physical examination revealed hyperkeratotic vegetative and hyperpigmented papules that diffusely involved the right lower extremity with a linear distribution (Fig. 1). Nail examination of the right hand showed white and red longitudinal bands and subungual hyperkeratosis (Fig. 2). There was no mucosal involvement. A biopsy specimen of a papule showed hyperkeratosis, papillomatosis, suprabasilar clefting with loss of cell adhesion (acantholysis), dyskeratosis (corps ronds and grains), and a chronic inflammatory dermal infiltrate (Figs. 3 & 4). These findings were consistent with the diagnosis of DD.

Figure 3Figure 4
Figure 3. Histological examination of a skin biopsy sample revealed acantholysis, dyskeratosis and chronic inflammatory dermal infiltrate (H&E, x40).
Figure 4. Same specimen as in Figure 3 showing acantholysis and dyskeratosis (H&E, x100).

The patient was treated with topical tretinoin cream to the affected areas. He obtained partial regression of the disease after two months of treatment.


Darier disease (keratosis follicularis) is an autosomally dominant inherited condition that is characterized by a symmetric eruption of keratotic, reddish brown papules that occur in seborrheic areas. Punctate keratoses or pits are frequently noted on the palms and soles. The nails show subungual hyperkeratosis, fragility, and splintering, with white and red alternating longitudinal bands and triangular nicking of the distal nail plate. The trunk is the most frequent site. The initial cutaneous findings generally develop after adolescence; sunlight or hot moist weather may provoke the condition [1]. Onset usually occurs before 30 years of age, but may occur later as in our case.

Darier disease in a unilateral or localized pattern was first reported at the turn of the last century. This variant often lacked other features that were associated with typical Darier disease and the skin lesions were usually confined to a limited area [1].

The lack of a family history and absence of other signs of DD, as well as the limited distribution of the lesions in many patients, are in favor of a nevoid origin. Starink and Woerdeman reported seven cases showing unilateral, linear, or zosteriform patterns, without other findings of DD and suggested the name acantholytic dyskeratotic epidermal nevus [2]. In a review of 167 biopsy specimens taken from patients with epidermal nevi, Su observed only two specimens (1.2%) with DD-like changes [3].

A singular report described localized DD affecting the scalp of a 5-year-old girl and of her mother, without other cutaneous abnormalities, possibly suggesting an autosomal dominant inheritance pattern [4]. Two other reports have associated typical localized DD with palmo-plantar papules and nail disorders characteristic of DD [5, 6].

The abnormal keratinocyte adhesion of DD is inherited in an autosomal dominant pattern, and the causative gene is located at chromosome 12, 12q23-24.1 [7]. The linear form of Darier disease could result from genetic mosaicism for this autosomal dominant disorder. This mutation occurs during embryogenesis [7, 8]. Two types of segmental mosaicism have been described recently. In the first type (type 1), the cutaneous manifestations show the same degree of severity that is found in the corresponding non-mosaic trait. In the second type (type 2), the segmentally involved lesions are much more severe and may be superimposed on a milder diffuse form of the same disease [8].

Our patient had a more pronounced involvement than the usual Darier disease lesions, disposed in a linear arrangement, with nail features. He probably presents a type 2 segmental manifestation of the disorder.


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