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Year : 2016  |  Volume : 2  |  Issue : 2  |  Page : 93-95

Acitretin therapy for inflammatory linear verrucous epidermal nevus

Department of Dermatotherapy and Cosmetology, Maharashtra Medical Foundation's Joshi Hospital, Pune, Maharashtra, India

Date of Web Publication20-Dec-2016

Correspondence Address:
Sharad D Mutalik
Planet Skin, 95-A-2, Samruddhi Apartments, Shivaji Nagar, Pune - 411 005, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2455-3972.196169

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Acitretin has been commonly used in the treatment of keratinization disorders due to its anti-inflammatory and anti-proliferative properties. ILVEN is a rare form of epidermal nevus with an early age of onset usually presenting as a localized itchy linear plaque resistant to treatment. The treatment of ILVEN aims at providing symptomatic and cosmetic improvement. We present two cases of ILVEN with solitary lesions treated with acitretin, with both patients showing remarkable reduction of the lesions with long term remission.

Keywords: Acetretin, ILVEN, systemic retinoids, verrucous epidermal nevus

How to cite this article:
Mutalik SD, Rasal Y. Acitretin therapy for inflammatory linear verrucous epidermal nevus. Indian J Drugs Dermatol 2016;2:93-5

How to cite this URL:
Mutalik SD, Rasal Y. Acitretin therapy for inflammatory linear verrucous epidermal nevus. Indian J Drugs Dermatol [serial online] 2016 [cited 2018 Apr 20];2:93-5. Available from: http://www.ijdd.in/text.asp?2016/2/2/93/196169

  Introduction Top

Inflammatory linear verrucous epidermal nevus (ILVEN) is a unilateral, benign, pruriginous, cutaneous hamartoma first described in literature in 1971. [1] Although the etiopathogenesis is not known exactly, it is thought to be associated with the upregulation of interleukin 1 and interleukin 6, tumor necrosis factor α, and intercellular adhesion molecule 1. [2]

Typically, ILVEN is resistant to treatment. Therapeutic options include topical and intralesional glucocorticoids, dithranol, calcipotriol, excision, cryotherapy, laser therapy, [2],[3] and systemic retinoids.

Systemic retinoids have been the treatment of choice for many disorders of keratinization. They possess anti-keratinizing, antiproliferative, and anti-inflammatory properties. We present our experience of acitretin therapy in 2 cases of ILVEN.

  Case reports Top

Case 1

An 18-year-old unmarried girl presented for evaluation of a pruritic, linear, plaque over her left-hand dorsa that appeared at 8 years of age with intermittent flare-ups and incomplete remission. The eruption and the associated pruritus did not respond to previous therapy with topical salicylic acid preparations and topical retinoids. The family history was not significant.

On dermatological examination, she had a linear crusted erythematous plaque over her dorsal left index finger extending over the adjacent web space and left-hand dorsa [Figure 1]. The rest of the physical examination was unremarkable, and no associated physical abnormality was found.
Figure 1: Inflammatory linear verrucous epidermal nevus lesion before acitretin therapy

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Results from the skin biopsy specimen from the lesion revealed an acanthotic epidermis with zones of parakeratosis devoid of granular cell layer alternating with zones of orthohyperkeratosis confirming the clinical diagnosis of ILVEN.

The patient was started on oral acitretin therapy after appropriate laboratory workup including fasting lipid profile and liver function tests (LFTs). A careful menstrual history and counseling regarding the importance of not becoming pregnant 1 month before, during therapy, and 3 years after therapy was impressed on the patient.

The patient was started on acitretin 25 mg PO daily (0.5 mg/kg) for 6 weeks and then tapered to 25 mg PO every other day for 12 weeks and then acitretin was stopped. Monthly monitoring of lipid profile and LFT was undertaken. There was a significant reduction in the hyperkeratosis and erythema at the end of the 4 weeks with almost complete clearance of the lesions at the end of therapy [Figure 2]. The patient remained lesion free for more than a year post discontinuation of acitretin. Subsequently, she lost to follow-up.
Figure 2: (a) Marked improvement at 4 weeks. (b) At 12 weeks with acitretin therapy

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Case 2

A 12-year-old boy presented with a pruritic, linear, hyperkeratotic, scaly plaque over the right forefoot dorsa since 4 years of age [Figure 3]. There was no family history of similar complaints.
Figure 3: Inflammatory linear verrucous epidermal nevus lesion over the right foot dorsum

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Cutaneous and systemic examination did not reveal any other abnormality. Histopathological examination confirmed the diagnosis of ILVEN.

His baseline investigations being normal, the patient received 25 mg PO of acitretin for 6 weeks with tapered dose of 25 mg every other day for another 12 weeks.

The lesion distinctly improved with a marked reduction in hyperkeratosis by 4 weeks into the treatment [Figure 4]. After discontinuation of therapy, the patient remained almost lesion free up to 1 year till he was lost to follow-up. The biochemical tests repeated at the end of treatment were normal.
Figure 4: Distinct improvement after 1 month of acitretin therapy

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Both patients did not have any significant adverse effect secondary to acitretin except for minimal skin dryness.

  Discussion Top

The introduction of retinoids in the armamentarium of dermatologic therapy has allowed many difficult-to-treat dermatoses to be now in a position of controllable diseases.

ILVEN is a rare chronic skin disorder believed to be resistant to therapy which may trouble the patient considerably. It has been shown to respond to a variety of antipsoriatic therapies, leading to some authors to believe that it is a nevoid form of psoriasis.

Acitretin is a second-generation monoaromatic retinoid used successfully in keratinization disorders in children. [4],[5],[6],[7] Anecdotal case reports have documented the efficacy of systemic retinoids for the treatment of ILVEN. Renner et al. have documented the disappearance of ILVEN lesions after 8 weeks of incremental acitretin (up to 30 mg) in a 36-year-old woman. [8]

In our case, long-standing ILVEN lesions in children resistant to topical first-line therapies responded very well to second-line oral therapy with acitretin with no major side effects and remained documented lesion free for up to a year after cessation of therapy. There are few reported case reports of acitretin use in ILVEN.

It is not clear whether ILVEN represents true form of inflammatory epidermal nevus. Review of literature suggests that some ILVEN cases respond to anti-psoriatic and anti-inflammatory treatment with only a slight reduction in itching and the inflammation. [9] These cases are due to an underlying (verrucous) epidermal naevus. In contrast, cases treated successfully with anti-inflammatory and anti-psoriatic therapy implicate that they have no underlying naevus. According to Hofer, these should rather be called inflammatory linear verrucous eruption (ILVE(N). [10]

We present these two case reports as an anecdotal evidence of successful judicious use of acitretin for the treatment of ILVEN.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Altman J, Mehregan AH. Inflammatory linear verrucous epidermal nevus. Arch Dermatol 1971;104:385-9.  Back to cited text no. 1
Zvulunov A, Grunwald MH, Halvy S. Topical calcipotriol for treatment of inflammatory linear verrucous epidermal nevus. Arch Dermatol 1997;133:567-8.  Back to cited text no. 2
Lee SH, Rogers M. Inflammatory linear verrucous epidermal naevi: A review of 23 cases. Australas J Dermatol 2001;42:252-6.  Back to cited text no. 3
Nazzaro V, Blanchet-Bardon C, Mimoz C, Revuz J, Puissant A. Papillon-Lefèvre syndrome. Ultrastructural study and successful treatment with acitretin. Arch Dermatol 1988;124:533-9.  Back to cited text no. 4
Katz HI, Waalen J, Leach EE. Acitretin in psoriasis: An overview of adverse effects. J Am Acad Dermatol 1999;41(3 Pt 2):S7-12.  Back to cited text no. 5
Lacour M, Mehta-Nikhar B, Atherton DJ, Harper JI. An appraisal of acitretin therapy in children with inherited disorders of keratinization. Br J Dermatol 1996;134:1023-9.  Back to cited text no. 6
Katugampola RP, Finlay AY. Oral retinoid therapy for disorders of keratinization: Single-centre retrospective 25 years' experience on 23 patients. Br J Dermatol 2006;154:267-76.  Back to cited text no. 7
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. 8
Menni S, Restano L, Gianotti R, Boccardi D. Inflammatory linear verrucous epidermal nevus in a child? Int J Dermatol 2000;39:30-40.  Back to cited text no. 9
Hofer T. Does inflammatory linear verrucous epidermal nevus represent a segmental type1/type2 mosaic of psoriasis? Dermatology 2006;212:103-7.  Back to cited text no. 10


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


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