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 Table of Contents  
Year : 2015  |  Volume : 2  |  Issue : 2  |  Page : 34-36

Type one segmental Darier's disease

1 Department of Dermatology and STD, Vinayaka Mission's Medical College and Hospital, Karaikal, Pondicherry, India
2 Department of Pathology, G. Kuppuswamy Naidu Memorial Hospital (GKNM), Coimbatore, Tamil Nadu, India

Date of Web Publication6-Jan-2016

Correspondence Address:
Dr. Arunprasath Palanisamy
Department of Dermatology and STD, Vinayaka Mission's Medical College and Hospital, Karaikal - 609 609, Pondicherry
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2349-6029.173414

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A 50-year-old woman presented with multiple pruritic hyperpigmented papules in a zosteriform pattern involving the abdomen and back on the left side and in a linear pattern involving the left arm and forearm of 2 years duration. Histopathology revealed striking suprabasal acantholysis and marked dyskeratosis in the form of corps ronds and grains, a picture consistent with Darier's disease (DD). Type one segmental DD involving multiple sites in zosteriform and linear patterns is an uncommon condition and is reported for its rarity.

Keywords: Darier′s disease (DD), linear, segmental, unilateral, zosteriform

How to cite this article:
Palanisamy A, Kandasamy S, Vadivel S, Kothandapany S. Type one segmental Darier's disease. Indian J Dermatopathol Diagn Dermatol 2015;2:34-6

How to cite this URL:
Palanisamy A, Kandasamy S, Vadivel S, Kothandapany S. Type one segmental Darier's disease. Indian J Dermatopathol Diagn Dermatol [serial online] 2015 [cited 2022 Jan 24];2:34-6. Available from: https://www.ijdpdd.com/text.asp?2015/2/2/34/173414

  Introduction Top

Darier's disease (DD) is an autosomal dominant disorder that is characterized clinically by multiple keratotic papules involving mainly the seborrheic areas and histologically by acantholysis and dyskeratosis. In contrast, segmental DD is characterized by localization of keratotic papules in a linear or zosteriform pattern following the  Lines of Blaschko More Details in a unilateral fashion. [1] A case of type one segmental DD presenting with zosteriform and linear lesions involving multiple sites is reported for its rare occurrence.

  Case Report Top

A 50-year-old woman presented with multiple pruritic hyperpigmented skin lesions involving the abdomen and back on the left side for 2 years. The lesions got aggravated during summer with a tendency for regression during the rest of the seasons. Family history was not contributory. Examination revealed multiple hyperpigmented papules and plaques in a zosteriform pattern involving the abdomen and back on the left side. Some of the papules had coalesced to form verrucous plaques on the back [Figure 1]. She also had hyperpigmented papules arranged in a linear pattern involving the lateral aspect of the left arm and flexor aspect of the left forearm [Figure 2]. Examination of nails and mucous membranes did not reveal any abnormality. She was a recently diagnosed case of rheumatoid arthritis and dyslipidemia on treatment with hydroxychloroquine, atorvastatin, and weekly methotrexate. A punch biopsy specimen was obtained from a plaque on the back and subjected to histopathological examination, which revealed hyperkeratosis, acanthosis, papillomatosis, and striking suprabasal acantholysis. Also, there was marked dyskeratosis in the form of corps ronds and grains with lymphocytic dermal infiltrate, a picture consistent with DD [Figure 3] and [Figure 4]. A diagnosis of type one segmental DD was entertained. The patient was prescribed topical emollient and tretinoin 0.025% cream. However, the patient was lost to follow-up after 2 months.
Figure 1: Hyperpigmented papules and plaques in a zosteriform pattern involving the left side of back

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Figure 2: Hyperpigmented papules arranged in a linear pattern involving the flexor aspect of the left forearm

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Figure 3: Hyperkeratosis, acanthosis, papillomatosis, suprabasal acantholysis, and dyskeratotic cells in the cleft [hematoxylin and eosin (H and E) ×40]

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Figure 4: (a) Suprabasalacantholysis and dyskeratosis in the form of corps ronds and grains (H and E ×100) (b) High power view of acantholytic lacuna with dyskeratotic cells (H and E ×400)

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  Discussion Top

DD was independently reported by Jean Darier and James C. White in 1889. [1] DD is an autosomal dominant disorder with complete penetrance and variable expressivity, characterized by keratotic and crusted papules and plaques involving mainly the seborrheic areas, palmoplantar pits, characteristic nail changes in the form of V-shaped nicking of distal nail plate and oral mucosal involvement. [1]

Its pathogenesis has been linked to mutations involving the ATP2A2 gene that codes for sarco/endoplasmic reticulum calcium ATPase isoform 2, a pump that transports calcium (Ca 2+ ) from cytosol to lumen of the endoplasmic reticulum, thus hampering the intracellular Ca 2+ signaling and keratinocyte intercellular adhesion and differentiation. [2]

A localized form of DD was first described by Kreibich in 1906. Nearly 10% cases of DD display a localized pattern often following Blaschko's lines. [3],[4] The localized variant is also referred to as zosteriform, linear, unilateral, or segmental DD and these may be localized to one half of the body. [3],[5] These patients develop skin lesions in the third or fourth decade of life, with a negative family history and without other associated features of DD. [6],[7] Our case had a similar manifestation.

Two patterns of segmental DD exist: In type 1 pattern, lesions follow Blaschko's lines unilaterally, whereas type 2 disease demonstrates focal areas of increased severity in patients with generalized DD. [1],[2],[3],[4] In the present case, the lesions were confined to the left side of the body, and occurrence of both zosteriform and linear patterns involving multiple segments was an interesting finding in this case.

Localized DD is considered a genetic mosaic of generalized DD resulting from a post-zygotic somatic mutation in early embryogenesis. [7],[8] The zosteriform pattern described in localized DD would actually exhibit a Blaschkoid rather than a dermatomal distribution. [8] Likewise, in this case the zosteriform lesions were found to follow the lines of Blaschko.

Histopathological findings of segmental DD do not show any distinct feature from that of generalized DD. [9] Histopathologial examination reveals a combination of acantholysis and dyskeratosis. Acantholysis tends to be suprabasal and two distinct types of dyskeratotic cells are appreciated: "corps ronds" and "grains."

Acantholytic dyskeratotic epidermal nevus (ADEN) is a condition that is clinically and histologically indistinguishable from segmental DD. Uncertainty prevails over using the terms ADEN or segmental DD. Munro and Cox reported a case of ADEN with characteristic nail and palmar changes of DD on the same side of the body. [10] Sakuntabhai et al. for the first time established mutations involving ATP2A2 gene in the affected skin of ADEN, the gene that is also defective in generalized DD and pointed out that these patients do have segmental DD and are mosaics for an ATP2A2 mutation. [11] The late age of onset, which would be unusual for epidermal nevi but is characteristic of DD supports the diagnosis of segmental DD. [6],[7] The precipitating factors in DD such as sunlight, heat, and sweating were implicated as an aggravating factor in many reported cases of localized DD. [6],[7] Citing the above specifics, the present case could be labeled as segmental DD rather than ADEN.

Treatment options for segmental DD mainly include topical therapies because of localized involvement. Avoiding precipitating factors such as sunlight and heat may improve the symptoms. Emollient containing urea or lactic acid helps in reducing crusting and irritation. Topical retinoids improve the lesions by reducing hyperkeratosis. [4],[7],[8] Successful treatment of segmental DD using ablative fractional laser resurfacing has been reported. [12]

In this case, a differential diagnosis of linear lichen planus, linear porokeratosis, nevus unis lateris, and Blaschkoid linear acquired inflammatory skin eruption were considered initially. However, histopathology established the diagnosis. Type 1 segmental DD, characterized by the occurrence of both zosteriform and linear patterns involving multiple segments is an extremely rare presentation and hence, is highlighted.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Hohl D. Darier disease and Hailey-Hailey disease. In: Bolognia JL, Jorizzo JL, Schaffer JV, editors. Bolognia Textbook of Dermatology. 3 rd ed. New York: Elsevier; 2012. p. 887-97.  Back to cited text no. 1
Burge S, Hovnanian A. Acantholytic disorders of the skin. In: Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, Wolff K, editors. Fitzpatrick′s Dermatology in General Medicine. 8 th ed. New York: McGraw-Hill; 2012. p. 550-62.  Back to cited text no. 2
Gilaberte M, Puig L, Vidal D, Alomar A. Acantholytic dyskeratotic naevi following Blaschko′s lines: A mosaic form of Darier′s disease. J Eur Acad Dermatol Venereol 2003;17:196-9.  Back to cited text no. 3
Lin FL, Hu SL, Liang CW. Seasonal recurrence of linear Darier′s disease - A case report. Dermatol Sinica 2008;26:236-41.  Back to cited text no. 4
Itin PH, Büchner SA, Happle R. Segmental manifestation of Darier disease.What is the genetic background in type 1 and type 2 mosaic phenotypes? Dermatology 2000;200:254-7.  Back to cited text no. 5
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. 6
O′Malley MP, Haake A, Goldsmith L, Berg D. Localized Darier disease. Implications for genetic studies. Arch Dermatol 1997;133:1134-8.  Back to cited text no. 7
Reese DA, Paul AY, Davis B. Unilateral segmental Darier disease following Blaschko lines: A case report and review of the literature. Cutis 2005;76:197-200.  Back to cited text no. 8
Ren V, Champion RW, Boyd AS, Powers JG. Linear, pruritic red to brown papules on the left chest. Dermatol Online J 2014;20. pii: 13030/qt8ww2w4xw.  Back to cited text no. 9
Munro CS, Cox NH. An acantholytic dyskeratotic epidermal naevus with the features of Darier′s disease on the same side of the body. Br J Dermatol 1992;127:168-71.  Back to cited text no. 10
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. 11
Krakowski AC, Nguyen TA, Eichenfield LF. Treatment of segmental keratosis follicularis (Darier disease) using ablative fractional laser resurfacing. Dermatol Surg 2015;41:516-8.  Back to cited text no. 12


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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