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Year : 2013  |  Volume : 58  |  Issue : 2  |  Page : 158
Inflammatory linear verrucous epidermal nevus in perineum and vulva: A report of two rare cases

Department of Dermatology, IPGMER and SSKM Hospital, Kolkata, West Bengal, India

Date of Web Publication5-Mar-2013

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Falguni Nag
Flat 1B, 356/22A, NSC Bose Road, Kolkata 700 047, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-5154.108078

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How to cite this article:
Nag F, Ghosh A, Surana TV, Biswas S, Gangopadhyay A, Chatterjee G. Inflammatory linear verrucous epidermal nevus in perineum and vulva: A report of two rare cases. Indian J Dermatol 2013;58:158

How to cite this URL:
Nag F, Ghosh A, Surana TV, Biswas S, Gangopadhyay A, Chatterjee G. Inflammatory linear verrucous epidermal nevus in perineum and vulva: A report of two rare cases. Indian J Dermatol [serial online] 2013 [cited 2021 Mar 4];58:158. Available from: https://www.e-ijd.org/text.asp?2013/58/2/158/108078

What was known? ILVEN is a rare form of epidermal nevus with an early age of onset with predilection for trunk and lower limb.

   Introduction Top

Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare form of epidermal nevus. It usually presents in childhood as an itchy linear plaque resistant to treatment. It is commonly found over buttocks and lower limbs. ILVEN in perineum and vulva is very rare. We hereby report two cases of ILVEN, one over the perineal region of a 24-year-old male and the other over the left vulva of a 4-year-old child, for its rarity and difficulties in diagnosis.

   Case Reports Top

Case 1

A 24-year-old male presented with a pruritic, macerated plaque mostly over left perineal region since the age of 5 years with intermittent flare-up and remission. The lesion extended from the perineal region to the scrotum and inner aspect of left thigh [Figure 1]. Thorough examination revealed no other abnormality anywhere in the body. Family history was not significant. Histopathology revealed hyperkeratosis with a characteristic alternating orthokeratosis, hypergranulosis with parakeratosis and agranulosis along with upperdermal lymphocytic infiltrate [Figure 2]. Diagnosis of ILVEN was thus made and treated with topical corticosteroid without any long term benefit.
Figure 1: Adult with ILVEN over left perineum

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Figure 2: Photomicrograph(×400, H and E stain) showing HPE of ILVEN in adult

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Case 2

A four year old girl presented with a severely pruritic scaly pigmented plaque over the left vulva since birth. It gradually extended to the groin, perineum, and gluteal region on the same side [Figure 3]. Cutaneous and systemic examination did not reveal any other abnormality. Histopathological examination was suggestive of ILVEN [Figure 4]. She was treated with topical calcipotriol with marked improvement.
Figure 3: Child with ILVEN over left vulva

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Figure 4: Photomicrograph(× 400, H and E stain) showing HPE of ILVEN in child

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   Discussion Top

ILVEN is a rare variant of epidermal nevus. Clinically it presents as pruritic, verrucous, linear, plaque with an early age of onset and is resistant to treatment. [1] In half of the cases the onset is in the first 6 months of age [2] and almost all are present by the age of 4 years. [1] The ages of onset in our cases were at 5 years (Case 1) and at birth (Case 2). ILVEN most commonly occurs over the lower limb with a predilection for the left buttock. [1],[2] Although literature search showed that inguino-genital presentation is very rare, both our cases presented with same. There have been further case reports of a 21-year-old male who had linear verrucous plaque over left side of body along with the involvement of penis and scrotum [3] and another male presenting with linear epidermolytic verrucous epidermal nevus over scrotum. [4] Surprisingly both of our cases had left sided lesions.

Histopathology of ILVEN is remarkable with columns of hypergranulosis, orthokeratosis alternating with agranulosis and parakeratosis with a dermal inflammatory infiltrate. [2] Both our cases showed strikingly similar histopathological features.

ILVEN over inguino-genital region is often misdiagnosed as candidiasis because of the macerated look, wart, [4] psoriasis [5] or even eczema. [5] Our first case was being treated for candidial intertrigo for long duration before visiting us. Likewise, the child was being treated as eczema with a waxing and waning course. Hence, a histopathological examination of doubtful persistent lesions should be carried out.

Epidermal nevus is sometimes associated with abnormalities of other systems mainly neurological, skeletal and ocular, giving rise to the epidermal nevus syndrome. [2] We did not get any systemic involvement in our small series, although it has been rarely reported with ILVEN.

Treatment of ILVEN is disappointing. Topical therapies like corticosteroid, [6] tretinoin and 5- fluorouracil, [7] calcipotriol [8] have been tried with variable results. Systemic retinoid have also been tried. [9] Surgical excision is done in refractory cases. [10]

The purpose of reporting these two cases is that, ILVEN should be suspected in persistent pruritic genital lesions not only in children but also in adults and biopsy be carried out for confirmation of diagnosis. The preponderance of left sided lesions in both of our cases as also reported in other studies could be studied further.

   References Top

1.Le K, Wong LC, Fischer G. Vulval and perianal inflammatory linear verrucous epidermal naevus. Australas J Dermatol 2009;50:115-7.  Back to cited text no. 1
2.Rogers M, McCrossin I, Commens C. Epidermal nevi and epidermal nevus syndrome: A review of 131 cases. J Am Acad Dermatol 1989;20:476-88.  Back to cited text no. 2
3.Sethuraman G, Khaitan BK, Tejasvi T, Das S, Manchanda Y, Sirka C, et al. Verrucous epidermal nevus with unusual features. Pediatr Dermatol 2006;23:98-9.  Back to cited text no. 3
4.Sarifakioglu E, Yenidunya S. Linear epidermolytic verrucous epidermal nevus of the male genitalia. Pediatr Dermatol 2007;24:447-8.  Back to cited text no. 4
5.Harth W, Linse R. Dermatological symptoms and sexual abuse: A review and case reports. J Eur Acad Dermatol Venereol 2000;14:489-94.  Back to cited text no. 5
6.Morag C, Metzker A. Inflammatory linear epidermal nevus: Report of seven new cases and review of literature. Pediatr Dermatol 1985;3:15-8.  Back to cited text no. 6
7.Kim JJ, Chang MW, Shwayder T. Topical tretinoin and 5-fluorouracil in the treatment of linear verrucous epidermal nevus. J Am Acad Dermatol 2000;43:129-32.  Back to cited text no. 7
8.Micali G, Nasca MR, Musumeci ML. Effect of topical calcipotriol on inflammatory linear verrucous epidermal nevus. Pediatr Dermatol 1995;12:386-7.  Back to cited text no. 8
9.Renner R, Rytter M, Sticherling M. Acitretin treatment of a systematized inflammatory linear verrucous epidermal naevus. Acta Derm Venereol 2005;85:348-50.  Back to cited text no. 9
10.Lee BJ, Mancini AJ, Renucci J, Paller AS, Bauer BS. Full-thickness surgical excision for the treatment of inflammatory linear verrucous epidermal nevus. Ann Plast Surg 2001;47:285-92.  Back to cited text no. 10

What is new? 1. ILVEN can have a late onset. 2. Rare presentation over perineum and vulva.


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


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