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LETTER TO EDITOR
Year : 2021  |  Volume : 22  |  Issue : 1  |  Page : 94-95

Bilateral Systematized Inflammatory Linear Verrucous Epidermal Nevus Treated with Topical and Systemic Retinoids: A Case Report


Department of Dermatology and Venereology and Leprosy, Government Medical College, Amritsar, Punjab, India

Date of Submission03-Apr-2010
Date of Decision08-Apr-2020
Date of Acceptance27-May-2020
Date of Web Publication31-Dec-2020

Correspondence Address:
Sandeep Sidhu
Department of Dermatology and Venereology and Leprosy, Government Medical College, C-13, Medical College Campus, Circular Road, Amritsar - 143 001, Punjab
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpd.IJPD_50_20

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How to cite this article:
Sidhu S, Mittal N, Kaur T. Bilateral Systematized Inflammatory Linear Verrucous Epidermal Nevus Treated with Topical and Systemic Retinoids: A Case Report. Indian J Paediatr Dermatol 2021;22:94-5

How to cite this URL:
Sidhu S, Mittal N, Kaur T. Bilateral Systematized Inflammatory Linear Verrucous Epidermal Nevus Treated with Topical and Systemic Retinoids: A Case Report. Indian J Paediatr Dermatol [serial online] 2021 [cited 2021 Apr 22];22:94-5. Available from: https://www.ijpd.in/text.asp?2021/22/1/94/305808



Sir,

Inflammatory linear verrucous epidermal nevus (ILVEN) is a sporadic, pruritic keratinocytic hamartoma and is resistant to treatment. Predominantly, it is unilateral; however, infrequently, it can be systematized.

A 9-year-old boy presented with the complaint of multiple hyperpigmented raised lesions covering almost the whole of the body associated with mild-to-moderate itching since the age of 6 months. Parents noticed the appearance of multiple raised hyperpigmented lesions on the bilateral knees, simultaneously which slowly increased in thickness and size to involve bilateral lower limb, extending to external genitalia, trunk, bilateral upper limb, and neck in this order over a period of next few months. There was no significant birth and family history, and the child was otherwise healthy with no developmental delay. He took a number of medications from various practitioners, not documented, without any significant improvement.

On examination, multiple confluent hyperpigmented papules and warty plaques in linear arrangement were present on the whole body, with relative sparing of scalp, face, palms, and soles. On systemic examination, hair, teeth, nails, and mucosae were normal. On dermoscopic evaluation, brownish scales with brown globules were seen [Figure 1]. Histopathologically, lesions showed orthohyperkeratosis, parakeratosis, mild papillomatosis, hypergranulosis, acanthosis and elongation of rete ridges, superficial perivascular and interstitial inflammatory infiltrate in the papillary dermis, suggestive of VEN [Figure 2]. Thorough evaluation and investigations (complete blood count, hepatic and renal profile, urine complete, lipid profile, viral markers, and ultrasound whole abdomen) were done. After clinicopathologic correlation, final diagnosis of systematized ILVEN was made. Retinoids in the form of topical tretinoin 0.04% at night time along with acitretin 10 mg (0.5 mg/kg) were started; however, due to monetary constraints, he was shifted to Vitamin A 50,000 IU daily with appropriate drug-free interval. The patient was followed up for another 2 months and showed marked improvement [Figure 3].
Figure 1: Dermoscopy showing brownish scales (white arrow) and brown circles (blue arrow) suggestive of verrucous epidermal nevus (Dino lite digital microscope AM7515MZT, polarized mode)

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Figure 2: Histopathology showing orthohyperkeratosis, parakeratosis, hypergranulosis, and acanthosis with an elongation of rete ridges. Superficial perivascular and interstitial inflammatory infiltrate was also seen (H and E, ×40)

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Figure 3: Lesions before the start of therapy (a) and 12 weeks posttreatment (b) – showing marked improvement

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Etiopathogenesis is unknown, but Happle proposed that ILVEN might result from the activity of a transposable element that is partly expressed and partly silenced during early phases of development.[1] Although ILVEN is benign in nature, it may be associated with aplasia cutis congenita, skeletal and dental anomalies, central nervous system involvement, ocular defects; malignant transformation can be seen rarely in the form of basal cell carcinoma, keratoacanthoma, squamous cell carcinoma, and Bowen's disease, thus highlighting the role of thorough evaluation, long-term follow-up, and need for better therapeutics.[2]

Systematized ILVEN is one of the rarely reported pathologies. A number of therapies have been tried but in vain. These include topical as well as intralesional steroids, tacrolimus, dithranol, 5-fluorouracil, and calcipotriol. Surgical management consisting of laser ablation, electrofulguration, cryotherapy, full depth chemical peels, and excision followed by skin grafting has been the mainstay of treatment for a few years now.[3]

Since the introduction of retinoids and their proven efficacy in keratinization disorders have paved the way for their exploration in ILVEN, a few have reported their efficacy in ILVEN. Our study further adds to the evidence of possible role of retinoids in the treatment of ILVEN. Our study further adds to the evidence of possible role of retinoids in the treatment of ILVEN.

To conclude, Vitamin A and systemic retinoids may have a therapeutic as well as preventive role in ILVEN.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Happle R. Transposable elements and the lines of Blaschko: A new perspective. Dermatology 2002;204:4-7.  Back to cited text no. 1
    
2.
Kumar CA, Yeluri G, Raghav N. Inflammatory linear verrucous epidermal nevus syndrome with its polymorphic presentation – A rare case report. Contemp Clin Dent 2012;3:119-22.  Back to cited text no. 2
[PUBMED]  [Full text]  
3.
Behera B, Devi B, Nayak BB, Sahu B, Singh B, Puhan MR. Giant inflammatory linear verrucous epidermal nevus: Successfully treated with full thickness excision and skin grafting. Indian J Dermatol 2013;58:461-3.  Back to cited text no. 3
[PUBMED]  [Full text]  


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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