|Year : 2000 | Volume
| Issue : 5 | Page : 268-269
Acantholytic dyskeratotic epidermal naevus on the scalp
BC Ravikumar, C Balachandra, K Ramnarayan
B C Ravikumar
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Ravikumar B C, Balachandra C, Ramnarayan K. Acantholytic dyskeratotic epidermal naevus on the scalp. Indian J Dermatol Venereol Leprol 2000;66:268-9
|How to cite this URL:|
Ravikumar B C, Balachandra C, Ramnarayan K. Acantholytic dyskeratotic epidermal naevus on the scalp. Indian J Dermatol Venereol Leprol [serial online] 2000 [cited 2020 Jul 9];66:268-9. Available from: http://www.ijdvl.com/text.asp?2000/66/5/268/4945
| Introduction|| |
Acantholytic dyskeratotic epidermal naevus (ADEN) is a localised skin disease with the clinical morphology and histological features of Darier's disease. However, it differs from the latter in its early age of onset, the absence of other features of Dariers disease and the negative family history in such patients.  We are reporting here one such case because of its rarity and also for its unusual involvement.
| Case Report|| |
A 34-year-old farmer presented with an asymptomatic skin coloured raised skin lesion over the vertex of 25 years duration. The lesion initially progressed and became stable since last 5-6 years. Occasionally the lesion would erode and bleed. His family history was negative for any similar condition. Previous treatments with topical steroids had been unsuccessful.
Examination revealed a linear plaque over the vertex slightly to the right side of midline with rough warty surface and dimension of 3 X 7 cms [Figure - 1]. There were no other abnormalities detected on examination of the remaining skin, the mucosae and the nails. Study of biopsy specimen from the lesion showed hyperkeratosis, acantholysis and suprabasal clefts. A few dyskeratotic cells and corps ronds were also seen in the stratum granulosum [Figure - 2]. With these clinical and histopathological findings, the diagnosis of "acantholytic dyskeratotic epidermal naevus" was made.
| Discussion|| |
Since Kreibich  in 1906 reported a case of localised Darier's disease, many more unilateral or zosteriform epidermal lesions with the clinical and histological features of Darier's disease have been reported. However, because of early age of onset, the absence of other features of Darier's disease and the negative family history in such patients. Starink and Woerdemann suggested the term acantholytic dyskeratotic epidermal naevus (ADEN).  Munro and Cox described a patient with unilateral acantholytic dyskeratotic epidermal naevus with typical nail and palm changes of Darier's disease on the same side of the body.  Another similar patient has been reported recently supporting the concept of genetic mosaicism between ADEN and Darier's disease due to a somatic mutation during embryogenesis.  Based on this theory, some authors still consider that at least some of these reported cases could be variants of Darier's disease.  However the theory remains conjectural as the mutated gene has not been demonstrated.
So far 41 such cases have been reported in the world literature. , Out of these, only 2 had similar family history and the average age at onset was 27 years with a wide range from infancy to 79 years. Trunk is the most common site involved (40%) and involvement of scalp is rare.  In the only case of ADEN reported so far from India, the site affected was the upper back. Besides being a rare disease, the presence of ADEN on the scalp makes the present case interesting.
| References|| |
|1.||Kreibich K, Zum Wesender Psorospermosis Darier. Arch Dermatol syphilol (Wien) 1906;80:367. |
|2.||Starink TM, Woerdemann MJ. Unilateral systematised keratosis follicularis; a varient of Dariers disease or an epidermal naevus (acantholytic dyskeratolic epidermal naevus). Br J Dermatol1981;105:207-14. |
|3.||Munro CS, Cox NH. An acantholytic dyskeratotic epidermal naevus with other features of Darter's disease on the same side of the body. Br J Dermatol 1992; 127:168-171. [PUBMED] |
|4.||Cambiaghi S, Brusasco A, Grimatt R, et al. Acantholytic dyskeratotic epidermal naevus as a mosaic form of Darter's disease. J Am Acad Dermatol 1995; 32: 284-286. |
|5.||O Mally MP, Haake A, Goldsmith L, et al. Localised Darier's disease: Implications for genetic studies. Arch Dermatol 1997 ; 133: 11341138. |
|6.||Niramala I, Jayaraman M, Janaki VR, et al. Acantholytic dyskeratotic epidermal naevus. Indian J Dermatol Venereol Leprol 1997; 63: 114-116. |
[Figure - 1], [Figure - 2]