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Year : 1997  |  Volume : 63  |  Issue : 2  |  Page : 114-116

Acantholytic dyskeratotic epidermal naevus

Correspondence Address:
Irene Nirmala

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Source of Support: None, Conflict of Interest: None

PMID: 20944291

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A 43-year old male presented with unilateral warty papular eruptions of 20 years duration, involving the left side of trunk, left upper and lower limbs. There were also whitish macules intermingled with them. Histological features were compatible with Darier's disease. There was no evidence of any family history or palmar pits and nail changes typical of Darier's disease. We are reporting this case as acantholytic dyskeratotic epidermal naevus representing a mosaic form of Darier's disease.

Keywords: Acantholytic dyskeratotic epidermal naevus, Darier′s disease

How to cite this article:
Nirmala I, Jayaraman M, Janaki V R, Boopalraj J. Acantholytic dyskeratotic epidermal naevus. Indian J Dermatol Venereol Leprol 1997;63:114-6

How to cite this URL:
Nirmala I, Jayaraman M, Janaki V R, Boopalraj J. Acantholytic dyskeratotic epidermal naevus. Indian J Dermatol Venereol Leprol [serial online] 1997 [cited 2020 May 28];63:114-6. Available from: http://www.ijdvl.com/text.asp?1997/63/2/114/4534

  Introduction Top

Since Kresbach in 1966 reported a case of Darier's disease in a localised and zosteriform pattern many more unilateral or zosteriform epidermal lesions with the clinical and histological features of Darier's disease have been reported.[1] Starink and Woerdmann[2] have reviewed such cases and concluded that the occurrence of cases in infancy, lack of family history, the absence of other features of Darrier's disease in any reported case, it is desirable to describe the localised lesion as acantholytic dyskeratotic epidermal naevus rather than localised Darier's disease. Munro and Cox[3] have also proposed that localised lesions displaying the clinical and histological features of Darrier's disease should be regarded as acantholytic dyskeratotic epidermal naevus and not Darier's disease of limited extent.

  Case Report Top

A 43-year-old male presented with asymptomatic crusted warty papular eruptions on one side of the body which he had noticed from the second decade of his life. There was no family history of similar skin lesions.

Examination revealed numerous hyperpigmented warty papules distributed along the  Lines of Blaschko More Details and in zosteriform patterns over the chest abdomen and back on the left side only [Figure - 1]. Similar lesions were noticed on the left upper limb including the dorsum of the hand and the left lower limb including the dorsum of the foot. Intermingled with the papules on the trunk were numerous depigmented macules. There were no oral lesions, no palmar pits and nails were normal.

Histological examination of a typical papule showed hyperkeratosis, irregular acanthosis, suprabasal clefts in some areas and corps ronds in upper stratum malpighi ([Figure - 2]a&[Figure - 2]b). Radiological examination of the long bones and skull was normal.

  Discussion Top

Is acantholytic dyskeratotic epidermal naevus a different entity or is it a localised form of Darier's disease? The lack of family history and other features of the disease like nail changes, oral mucosal changes and palmar pits seem to classify it as a separate entity yet the clinical appearance of the warty papules, the histologic and microscopic features of both the conditions are the same. Some forms of acantholytic dyskeratotic epidermal naevus could represent a mosaic form of Darier's disease due to a somatic mutation during embryogenesis.[4] This is particularly true for acantholytic dyskeratotic epidermal naevus following Blaschko's lines.[5] In mosaic forms of Darier's disease it has been suggested that the presence of manifestations like palmar pits, nail dystrophy and mucosal involvement is related to the time of onset of mutation in embryogenesis; the earlier the onset of mutation in embryogenesis the wider the area involved by the disease. Our patient who showed crusted papular eruptions following the lines of Blaschko on one side of the body without evidence of palmar pits, nail changes and oral mucosal changes supports this argument.

Since our patient developed the disease during the second decade, exhibited macules of leukoderma reported in Darier's disease along with the characteristic warty papules which showed typical histologic features, we report our patient as an example of acantholytic dyskeratotic epidermal naevus representing a mosaic form of Darier's disease.

  References Top

1.Leeming JAL. Acquired linear naevus showing histological features of keratosis follicularis. Br J Dermatol 1969;81:128-31.  Back to cited text no. 1    
2.Starink THM, Woerdeman MJ. Unilateral systematized keratosis follicularis : a variant of Darier's disease or an epidermal naevus (acatholytic dyskeratotic epidermal naevus). Br J Dermatol 1981;105:207-14.  Back to cited text no. 2    
3.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. 3  [PUBMED]  
4.Cambiaghi S, Brusasco A, Crimalt R, Caputo R. Acantholytic dyskeratotic epidermal naevus as a mosaic form of Darier's disease. J Am Acad Dermatol 1995;32:284-6.  Back to cited text no. 4    
5.Jackson R. The lines of Blaschko : a review and reconsideration. Br J Dermatol 1976;95:349-60.  Back to cited text no. 5  [PUBMED]  


[Figure - 1], [Figure - 2]


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