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Year : 2015  |  Volume : 6  |  Issue : 5  |  Page : 345-347  

Linear Darier's disease: A case with bilateral presentation

Department of Dermatology, Gauhati Medical College and Hospital and Dr. K.N.Barua Institute of Dermatological Sciences (BIDS), Guwahati, Assam, India

Date of Web Publication4-Sep-2015

Correspondence Address:
Anal Jyoti Bordoloi
Department of Dermatology, Gauhati Medical College and Hospital, Guwahati - 781 032, Assam
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Source of Support: Nil, Conflict of Interest: None declared.

DOI: 10.4103/2229-5178.164485

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Darier's disease is an autosomal dominant genodermatosis characterized by a persistent eruption of hyperkeratotic greasy papules mainly over the seborrheic sites of the body, usually associated with nail abnormalities and sometimes with mucous membrane lesions. The lesions typically occur in the younger age group and are associated with pruritus. We report here an atypical case of Linear Darier's disease with bilateral presentation in a middle-aged adult that could be confused with conditions such as lichen planus, inflammatory linear verrucous epidermal nevus, and wart.

Keywords: Darier's disease, genetic mosaicism, linear

How to cite this article:
Bordoloi AJ, Barua KN. Linear Darier's disease: A case with bilateral presentation. Indian Dermatol Online J 2015;6:345-7

How to cite this URL:
Bordoloi AJ, Barua KN. Linear Darier's disease: A case with bilateral presentation. Indian Dermatol Online J [serial online] 2015 [cited 2022 Jan 18];6:345-7. Available from: https://www.idoj.in/text.asp?2015/6/5/345/164485

   Introduction Top

Darier's disease, also known as Darier–White disease or keratosis follicularis is an autosomal dominant dermatoses characterized by warty, hyperkeratotic papules that are particularly distributed over the seborrheic sites of the body. It may be associated with nail dystrophy, palmoplantar pits, "cobblestoning" of oral mucosa[2],[11] and sometimes with neuropsychiatric abnormalities. The condition was first described by Darier and White in 1889. The average age of onset is usually during childhood and adolescence, with no gender predilection. Darier's disease in a localized pattern was first described in 1906 by Kreibich and since then various clinical variants have been described in the form of unilateral, linear, segmental, or zosteriform Darier's disease. The term "Acantholytic Dyskeratotic Epidermal Nevus" is used synonymously with linear Darier's disease.[4]

   Case Report Top

A 55-year-old male patient presented to our clinic with the chief complaint of recurrent, pruritic eruption over his scalp, forehead, face, and neck region since the last seven years. The eruption first appeared on the right side of his forehead as some linearly distributed papules and had remained stable for about five years without any symptoms due to which the patient did not seek any medical advice. But since then they spread in a linear pattern to involve the right side of his face, neck, and scalp as well as to the left side of his forehead and neck. There was associated pruritus and the lesions aggravated on sun exposure. He also complained of loss of hair from the scalp over the areas where these lesions appeared. There was no history of similar lesions in the family.

On examination, hyperkeratotic, skin colored to hyperpigmented, grouped as well as scattered papules arranged in a linear pattern were seen involving the right side of his forehead, right cheek, and left side of forehead [Figure 1]a, [Figure 1]b and [Figure 1]c. Some of the lesions had a violaceous hue but without any whitish streaks on the surface. A similar distribution was also seen on right side of his neck [Figure 1]d. These were not associated with erythema or scaling. On further examination, a few hyperkeratotic papulonodular eruptions were seen over the vertex of his scalp [Figure 1]e. The surrounding skin showed some pigmented macules and was slightly atrophic with loss of hair.
Figure 1: (a) Linear grouped hyperkeratotic and violaceous papules over forehead. (b) Similar lesions on the face. (c) Lesions on left side of forehead. (d) Lesions over neck. (e) Lesions over scalp

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Nail examination did not show any abnormality and the oral mucosa was also normal.

With the differential diagnosis of inflammatory linear verrucus epidermal nevus (ILVEN), linear lichen planus, warts, seborrheic keratosis, and linear Darier's disease we did a punch biopsy from the lesional skin over the forehead and the scalp. The histopathological examination revealed a circumscribed area of hyperkeratosis with acantholytic dyskeratosis and suprabasal cleft formation. Within the focus, the epidermis showed scattered acantholytic dyskeratotic cells (corps ronds) [Figure 2]a and [Figure 2]b. The stratum corneum showed an array of parakeratotic dyskeratotic cells (grains). The underlying dermis showed sparse superficial perivascular lymphohistiocytic infiltrate. With the above findings, a diagnosis of "linear Darier's disease" was made. The patient was at first started on topical retinoic acid (0.05%) to be applied once in the evening for one month. However, as he did not show any significant improvement, he was started on a course of oral isotretinoin 20 mg daily to which his skin lesions responded.
Figure 2: (a) Histopathology showing suprabasal cleft formation, acantholysis, and parakeratotic dyskeratotic cells, H and E, ×10. (b) High power magnification showing corps-ronds and grains, H and E, ×40

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   Discussion Top

Darier's disease, a dominantly inherited skin condition, is characterized by hyperkeratotic papules coalescing to form warty plaques on symmetrical areas of the face, trunk, and extremities. Mutations of the ATP2A2 gene located in chromosome 12 encoding SERCA2, which has an important role in calcium signal transduction, have been identified as the molecular basis of this condition.[1] It has been found that selective inhibition of SERCA pumps interfere with the formation of intercellular junctions and cellular adhesion.

The localized form of Darier's disease is rare and was first described by Kreibich in 1906.[2] This variant often lacked other features that were associated with typical Darier disease and the skin lesions were usually confined to a limited area. It has been postulated to reflect genetic mosaicism occurring during embryogenesis. It has been estimated that in 10% of cases of Darier's disease, the lesions are distributed in a linear pattern following the  Lines of Blaschko More Details. The most commonly affected sites are the trunk and the limbs. A family history is usually absent and rarely other associated features of Darier's disease have been reported.[3]

Aggravating factors are the same as seen in diffuse Darier's disease like light, heat, sweating, and friction. Pregnancy has also been shown to exacerbate this condition. The onset of the disease is usually in the third or fourth decade of life and there is no sexual predilection. Starink and Woerdeman reported seven cases showing unilateral, linear, or zosteriform patterns, without other findings and suggested the name "Acantholytic Dyskeratotic Epidermal Nevus."[4] Munro and Cox described a patient who had unilateral acantholytic dyskeratotic skin lesions with nail and palm changes of Darier's disease.[5] According to Happle's classification of cutaneous mosaicism for genetic disorders, segmental Darier's disease is of two types. Type 1 form retains the heterozygosity of mutation and the skin outside the segmental lesion is normal, whereas Type 2 is rarer, more severe, and is characterized by linear streaks over a background of generalized Darier's disease.[6],[7],[8] Our case probably belonged to the Type 1 form because it was localized and the surrounding skin was also normal.

Involvement of the scalp in linear Darier's disease has been rarely reported in the literature.[9],[10] Moreover, the bilateral involvement of the linear variant as was seen in our case is also a striking feature, which has not been reported, to the best of our knowledge. A recurrent form of Linear Darier's Disease aggravated by sunlight was reported by Plantin et al.[11]

Treatment modalities include topical agents such as salicylic acid, lactic acid, and retinoids such as retinoic acid and tazarotene.[13] Systemic retinoids mainly isotretinoin have also been shown to be effective. If medical therapy is ineffective, carbon dioxide laser and erbium: YAG laser have been used for chronic, recalcitrant cases.[14],[15]

Our patient, a middle-aged individual, had lesions distributed over the face and neck, which is quite uncommon for the linear variant of Darier's disease. Other features such as the presence of bilateral lesions, involvement of the scalp, and the lack of the typical "dirty warty" look of the papules made this an unusual presentation.

   References Top

Sakuntabhai A, Dhitavat J, Burge S, Hovnanian A. Mosaicism for ATP2A2 mutations causes segmental Darier's disease. J Invest Dermatol 2000;115:1144-7.  Back to cited text no. 1
Lin F, Hu S, Liang C. Seasonal recurrence of linear Darier's disease: A case report. Dermatol Sinica 2008;26:236-41.  Back to cited text no. 2
O'Malley MP, Haake A, Goldsmith L, Berg D. Localized Darier's disease: Implications for genetic studies. Arch Dermatol 1997;133:1134-8.  Back to cited text no. 3
Starink TM, Woerdeman MJ. Unilateral systematized keratosis follicularis. A variant of Darier's disease or an epidermal naevus (acantholytic dyskeratotic epidermal naevus)? Br J Dermatol 1981;105:207-14.  Back to cited text no. 4
Munro CS, Cox NH. An acantholytic dyskeratotic epidermal naevus with other features of Darier's disease on the same side of the body. Br J Dermatol 1992;127:168-71.  Back to cited text no. 5
Happle R. A rule concerning the segmental manifestation of autosomal dominant skin disorders: Review of clinical examples providing evidence for dichotomous type of severity. Arch Dermatol 1997;133:1505-9.  Back to cited text no. 6
Meziane M, Chraibi R, Kihel N, Hassam B, Karima S. Linear Darier disease. Dermatol Online J 2008;14:11.  Back to cited text no. 7
Boente Mdel C, Frontini Mdel V, Primc NB, Asial RA. Linear Darier disease in two siblings. An example of loss of heterozygosity. Ann Dermatol Venereol 2004;131:805-9.  Back to cited text no. 8
Wheeland RG, Donaldson ML, Bulmer GS. Localized Darier's disease of the scalp complicated by Trichophyton tonsurans infection. Arch Dermatol 1985;121:905-7.  Back to cited text no. 9
Ghorpade AK. Unilateral Blaschkoid Darier's disease over the forehead. Indian J Dermatol Venereol Leprol 2014;80:189-90.  Back to cited text no. 10
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Plantin P, Le Noac'h E, Leroy JP, Gourcuff H. Localized recurrent light-induced Darier disease following Blaschko lines. Ann Dermatol Venereol 1994;121:393-5.  Back to cited text no. 11
Gupta LK, Garg A, Khare AK, Mittal A. A case of zosteriform Darier's disease with seasonal recurrence. Indian Dermatol Online J 2013;4:219-21.  Back to cited text no. 12
[PUBMED]  Medknow Journal  
Brazzelli V, Prestinari F, Barbagallo T, Vassallo C, Agozzino M, Borroni G. Linear Darier's disease successfully treated with 0.1% tazarotene gel "short contact" therapy. EurJ Dermatol 2006;16:59-61.  Back to cited text no. 13
Beier C, Kaufmann R. Efficacy of erbium: YAG laser ablation in Darier disease and Hailey-Hailey disease. Arch Dermatol 1999;135:423-7.  Back to cited text no. 14
McElroy JA, Mehregan DA, Roenigk RK. Carbon dioxide laser vaporization of recalcitrant symptomatic plaques of Hailey-Hailey disease and Darier's disease. J Am Acad Dermatol 1990;23:893-7.  Back to cited text no. 15


  [Figure 1], [Figure 2]

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