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Year : 2015  |  Volume : 16  |  Issue : 4  |  Page : 255-257

Linear epidermolytic verrucous epidermal nevus with lichen planus: A rare case report

Department of Dermatology, Venereology and Leprology, Government Medical College, Kota, Rajasthan, India

Date of Web Publication24-Sep-2015

Correspondence Address:
Suresh Kumar Jain
Department of Dermatology, Venereology and Leprology, Government Medical College, Kota - 324 001, Rajasthan
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2319-7250.165639

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Verrucous epidermal nevi are congenital, noninflammatory, cutaneous hamartomas composed of keratinocytes. To the best of our knowledge, only single case of lichen planus (LP) arising in an inflammatory linear verrucous epidermal nevus has been reported. We herewith report a case of 10-year-old boy who developed LP over linear epidermolytic verrucous epidermal nevus.

Keywords: hamartoma, inflammatory linear verrucous epidermal nevus, lichen planus, verrucous epidermal nevi

How to cite this article:
Mehta P, Kumar R, Sharma M, Vyas K, Jain SK. Linear epidermolytic verrucous epidermal nevus with lichen planus: A rare case report. Indian J Paediatr Dermatol 2015;16:255-7

How to cite this URL:
Mehta P, Kumar R, Sharma M, Vyas K, Jain SK. Linear epidermolytic verrucous epidermal nevus with lichen planus: A rare case report. Indian J Paediatr Dermatol [serial online] 2015 [cited 2020 Sep 19];16:255-7. Available from: http://www.ijpd.in/text.asp?2015/16/4/255/165639

  Introduction Top

Epidermal nevus usually occurs as linear verrucous plaques composed of confluent keratotic papules. It may be present at birth or may appear during infancy. The condition persists and is resistant to therapy. Histologically, epidermal nevi show epidermoid keratinization, with varying proportions of hyperkeratosis, hypergranulosis, acanthosis, and papillomatosis with or without inflammation. Rarely, epidermolytic hyperkeratosis (EH), cornoid lamellation, dyskeratosis, or psoriasiform features are seen. Lichenoid epidermal nevus [1] and psoriasiform epidermal nevus [2] (also known as inflammatory verrucous epidermal nevus) have previously been described.

  Case report Top

A 10-year-old boy presented with a linear velvety brownish papules over the right thorax extending from the right lower anterolateral chest to posterior chest along the blaschko line [Figure 1]. It was present from birth and increased in size as the age progressed. There was no erythema or itching over these lesions. Patient also had a single, well-defined, nearly rounded, 4 cm × 2 cm sized violaceous plaque with overlying wickham striae present over anterior part of linear lesion. This lesion was itchy and was present since 2 months. A provisional diagnosis of linear verrucous epidermal nevus with lichen planus (LP) was kept, and biopsy was taken from both types of lesion. Systemic examination was within normal limit.
Figure 1: Verrucous epidermal nevus with lichen planus

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Histopathology of verrucous lesions showed epidermal hyperkeratosis, acanthosis and papillomatosis and perinuclear vacuolization of the keratinocytes that were consistent with the diagnosis of epidermolytic verrucous epidermal nevus while the violaceous plaque revealed features of LP [Figure 2] and [Figure 3]. Prognosis and course of lesion present by birth were explained to parents and intralesional triamcinolone actinide 5 mg/ml was given to LP lesion. By next follow-up, this lesion became flat and itching was improved. During follow-up of 4 months, no new lesion of LP was noticed.
Figure 2: Hyperkeratosis, acanthosis, papillomatosis and perinuclear vacuolization of stratum malpighi in histopathologic section from epidermal nevus (×10)

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Figure 3: Lichenoid infiltration and vacuolar degeneration of basement membrane in histopathologic section from lichen planus lesion (×40)

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  Discussion Top

Verrucous epidermal nevi are congenital, noninflammatory, cutaneous hamartomas composed of keratinocytes and caused by somatic mosaicism. At birth, they have a white, macerated appearance but within a few days take the form of pink or slightly pigmented, velvety streaks or plaques. Later, they darken and become more warty, sometimes with an erythematous base. The two variants epidermolytic and nonepidermolytic verrucous epidermal nevi are sometimes indistinguishable except on histology by the presence of perinuclear vacuolization of the keratinocytes in former.

The histological similarity between epidermolytic verrucous epidermal nevi and autosomal dominant bullous ichthyosiform erythroderma (BIE) led to the idea that the former represents a clone of cells expressing a post zygotic mutation in one of the BIE genes keratin 1 (KRT1) and KRT10. This was confirmed by finding mutations in KRT10 and KRT1 in certain studies. [3],[4],[5] It has been often hypothesized that epidermal nevi with EH reflect differentiation specific, suprabasal KRT1 and KRT10 gene mutations. [5] Epidermolytic verrucous epidermal nevi are not associated with extracutaneous abnormalities because KRT genes are expressed only in epithelia.

On the other side, LP is thought to be an immunologically mediated disorder [6] clinically characterized by shiny, violaceous, flat-topped polygonal papules which retain the skin lines, and which vary in size from pinpoint to a centimeter. Histopathology is diagnostic and shows hyperkeratosis, hypergranulosis, "saw-toothing" of the rete ridges, vacuolar degeneration of the basal layer and a dense band-like upper dermal infiltrate consisting predominantly of mononuclear cells and pigmentary incontinence. Schofield et al. found quantitative reduction in KRT 1 and 10 production along with increase expression of KRTs 6, 16 and 17 in LP. [7]

Thus, both verrucous epidermal nevus and LP have same pathogenesis of mutation or decrease expression of KRT 1 and/or KRT 10 gene, and this may explain the development of LP lesion over verrucous epidermal nevus.

In conclusion, presence of LP over linear epidermolytic verrucous epidermal nevus is a rare case and favors the role of aberrant KRT expression in the development of LP.

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Conflicts of Interest

There are no conflicts of interest.

  References Top

Brownstein MH, Silverstein L, Lefing W. Lichenoid epidermal nevus: "Linear lichen planus". J Am Acad Dermatol 1989;20:913-15.  Back to cited text no. 1
Altman J, Mehregan AH. Inflammatory linear verrucose epidermal nevus. Arch Dermatol 1971;104:385-9.  Back to cited text no. 2
Paller AS, Syder AJ, Chan YM, Yu QC, Hutton E, Tadini G, et al. Genetic and clinical mosaicism in a type of epidermal nevus. N Engl J Med 1994;331:1408-15.  Back to cited text no. 3
Moss C, Jones DO, Blight A, Bowden PE. Birthmark due to cutaneous mosaicism for keratin 10 mutation. Lancet 1995;345:596.  Back to cited text no. 4
Tsubota A, Akiyama M, Sakai K, Goto M, Nomura Y, Ando S, et al. Keratin 1 gene mutation detected in epidermal nevus with epidermolytic hyperkeratosis. J Invest Dermatol 2007;127:1371-4.  Back to cited text no. 5
Dutz JP. T-cell-mediated injury to keratinocytes: Insights from animal models of the lichenoid tissue reaction. J Invest Dermatol 2009;129:309-14.  Back to cited text no. 6
Schofield JK, De Berker D, Milligan A, Rohloff W, Marren P, Davies MG, et al. Keratin expression in cutaneous lichen planus. Histopathology 1995;26:153-8.  Back to cited text no. 7


  [Figure 1], [Figure 2], [Figure 3]


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