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Year : 2018  |  Volume : 19  |  Issue : 3  |  Page : 266-268

Systematized inflammatory linear verrucous epidermal nevus moderately responsive to systemic acitretin and topical calcipotriol

Department of Dermatology and Venereology, All India Institute of Medical Sciences, New Delhi, India

Date of Web Publication28-Jun-2018

Correspondence Address:
Dr. Savita Yadav
Department of Dermatology and Venereology, All India Institute of Medical Sciences, New Delhi
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpd.IJPD_53_17

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Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare form of epidermal nevus which usually presents in childhood, mostly on the lower half of the body in a blaschkoid distribution. A wide variety of topical, systemic, and surgical modalities have been tried for its treatment, but it is known to be refractory to treatment. We hereby are reporting a rare case of extensive systematized ILVEN which showed moderate response to treatment with acitretin and topical calcipotriol.

Keywords: Acitretin, calcipotriol, inflammatory linear verrucous epidermal nevus, systematized

How to cite this article:
Singh S, Rai M, Bhari N, Yadav S. Systematized inflammatory linear verrucous epidermal nevus moderately responsive to systemic acitretin and topical calcipotriol. Indian J Paediatr Dermatol 2018;19:266-8

How to cite this URL:
Singh S, Rai M, Bhari N, Yadav S. Systematized inflammatory linear verrucous epidermal nevus moderately responsive to systemic acitretin and topical calcipotriol. Indian J Paediatr Dermatol [serial online] 2018 [cited 2020 Oct 25];19:266-8. Available from: https://www.ijpd.in/text.asp?2018/19/3/266/217481

  Introduction Top

Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare form of epidermal nevus. It usually presents in childhood, sometimes at birth as an itchy, linear, verrucous, and hyperkeratotic erythematous plaque which is resistant to medical treatment. Clinically, it appears inflammatory and psoriasiform, with regular acanthosis on histopathology. Unlike noninflammatory epidermal nevi, ILVEN is far less common. It is usually unilateral and most often located on the lower half of the body, with a linear distribution that follows the  Lines of Blaschko More Details. We hereby report a case of extensive systematized ILVEN.

  Case Report Top

A 4-year-old female child presented with a history of progressively developing multiple verrucous hyperpigmented papules and plaques in generalized distribution since the age of 1 year. Parents noticed sudden development of barely elevated hyperpigmented lesions at multiple sites simultaneously, which slowly increased in thickness as well as the extent and was associated with moderate-to-severe itching. The child was born from nonconsanguineous marriage, and there was no similar history in the family. Her growth and development were normal for age. There was no history suggestive of any systemic illness. Before presenting to us, the patient was treated with undocumented topical medications without any improvement. On examination, there was generalized involvement in the form of discrete to confluent multiple hyperpigmented hyperkeratotic papules and plaques in blaschkoid distribution with mild underlying erythema in most of the lesions [Figure 1]. There was relative sparing of anterior trunk. Systemic examination was within normal limit. A punch biopsy (4 mm) taken from lesion over back showed marked compact hyperkeratosis alternating with parakeratosis, papillomatosis, and irregular acanthosis of the epidermis with upper dermal perivascular chronic inflammation and fair number of melanophages [Figure 2]. A final diagnosis of systematized ILVEN was considered on the basis of clinicopathological correlation. She was given acitretin 25 mg every alternate day along with topical application of calcipotriol ointment once daily and antihistamines. There was moderate improvement in itching, thickness, and size of lesions at the end of 5 months [Figure 3]. The dose of acitretin has been further reduced to 25 mg twice per week without any worsening of disease.
Figure 1: Multiple, well-defined keratotic papules and plaques with mild underlying erythema in blaschkoid distribution over back and posterior thigh

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Figure 2: Histopathology showing alternate ortho- and para-keratosis along with lichenoid interface changes (H and E, ×200)

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Figure 3: Moderate flattening of lesions at the end of 5 months

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  Discussion Top

ILVEN is a variant of verrucous epidermal nevus characterized by pruritic, erythematous scaly epidermal nevus which follows the Blaschko's lines. The exact pathogenesis is not known, but it is postulated that these disorders are due to the action of a lethal gene surviving by mosaicism, a concept that is hypothesized to explain the origin of several sporadic syndromes characterized by a mosaic distribution of skin defects.[1] The clinical criteria for the diagnosis of ILVEN were given by Altman and Mehregan which includes early age of onset, predominance in females, frequent involvement of the left leg, pruritus, distinctive psoriasiform appearance, and marked refractoriness to therapy.[2],[3] Most of the cases are seen to appear before the age of 5 years. However, adult onset of the condition has also been reported. Females are seen to be affected 4 times more commonly than males, but one study has found male preponderance.[2] Most common site of involvement is lower extremities, with the left side being more commonly affected than the right.[2] Most cases are sporadic although familial cases have been described.

The characteristic histopathology findings of ILVEN are sharply demarcated alternating areas of hypergranulosis with overlying orthokeratotic hyperkeratosis and hypogranulosis with overlying parakeratosis. Psoriasiform epidermal hyperplasia with spongiosis, exocytosis, and even microabscess formation may be seen. An upper dermal perivascular inflammatory infiltrate composed of lymphocytes and histiocytes is a regular feature; this infiltrate may extend to the overlying irregularly hyperplastic epidermis.

Clinically, it may be difficult to differentiate it from various linear and segmental dermatoses, i.e., linear and nevoid psoriasis, lichen striatus, linear Darier disease, linear porokeratosis, linear lichen planus, and the verrucous stage of incontinentia pigmenti. In such circumstances, diagnosis mainly depends on clinicopathological correlation.

Systematized ILVEN has been rarely described in the literature so far.[2],[4] Lapidoth et al. in their retrospective study evaluated the response of cryotherapy in 71 patients of VEN. Of 71 cases, there were six cases of systematized VEN. Small localized VEN (43 patients) required relatively few treatments and exhibited the best response while systematized VEN patients responded poorly.[5] Renner et al. used acitretin with good result in their patient.[2] We also found a similar result in our case with the combination of topical calcipotriol and oral acitretin. Bogle et al. used etanercept in their patient with significant improvement.[4]

Although a large number of treatment modalities are tried, ILVEN is known to be refractory to treatment. Potent topical steroids and intralesional steroids are seen to provide temporary symptomatic relief. Mutasim showed successful treatment of ILVEN with tacrolimus and fluocinonide combination.[3] Other topical treatments found to be effective are dithranol, retinoids, 5-fluorouracil, and calcipotriol. Laser ablation, electrofulguration, cryotherapy, medium to full-depth chemical peels, and full-thickness skin excision accompanied by skin grafting has been used as alternative surgical modalities.[6]

Thus, hereby, we are reporting a very unusual case of ILVEN in systematized manner in linear and blaschkoid distribution which showed a good response to topical calcipotriol ointment and oral acitretin.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Happle R. Lethal genes surviving by mosaicism: A possible explanation for sporadic birth defects involving the skin. J Am Acad Dermatol 1987;16:899-906.  Back to cited text no. 1
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. 2
Mutasim DF. Successful treatment of inflammatory linear verrucous epidermal nevus with tacrolimus and fluocinonide. J Cutan Med Surg 2006;10:45-7.  Back to cited text no. 3
Bogle MA, Sobell JM, Dover JS. Successful treatment of a widespread inflammatory verrucous epidermal nevus with etanercept. Arch Dermatol 2006;142:401-2.  Back to cited text no. 4
Lapidoth M, Israeli H, Ben Amitai D, Halachmi S. Treatment of verrucous epidermal nevus: Experience with 71 cases. Dermatology 2013;226:342-6.  Back to cited text no. 5
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. 6
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