Indian J Dermatol. 2013 Nov-Dec; 58(6): 461–463.

PMCID: PMC3827519
doi: 10.4103/0019-5154.119959 PMID: 24249899

Giant Inflammatory Linear Verrucous Epidermal Nevus:

Successfully Treated with Full Thickness Excision and Skin
Grafting
Binodini Behera, Basanti Devi, Bibhuti B Nayak,1 Bharti Sahu, Bhabani Singh, and Manas R Puhan

From the Department of Skin and VD, SCB Medical College, Cuttack, India
1Department of Plastic Surgery, SCB Medical College, Cuttack, India
Address for correspondence: Dr. Bharti Sahu, Department, of Skin and VD, SCB Medical
College, Cuttack, Odisha, India. E-mail: drbharati123@gmail.com
Received 2013 Jun; Accepted 2013 Aug.
Copyright : © Indian Journal of Dermatology
This is an open-access article distributed under the terms of the Creative Commons
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Abstract
Inflammatory linear verrucous epidermal nevus (ILVEN) is a pruritic,
erythematous scaly epidermal nevus which follows a Blaschko's
lines. Lichen striatus, linear Darier disease, linear porokeratosis,
linear lichen planus, linear psoriasis, and the verrucous stage of
incontinentia pigmenti may all have similar clinical presentations as
the linear verrucous epidermal nevus. ILVEN can be distinguished
from true nevoid psoriasis by pruritus and lack of response to
antipsoriatic treatments. Various therapeutic modalities have been
described, but no one therapy has been successful consistently.
Though giant ILVEN is a relative contraindication to surgical
excision, here we report a case showing effectiveness of full
thickness excision and skin grafting for this condition.

Keywords: Full thickness excision and skin grafting, inflammatory

linear verrucous epidermal nevus, nevoid psoriasis

Introduction
Introduction

What was known?

Inflammatory linear verrucous epidermal nevus (ILVEN) is a

pruritic, erythematous scaly epidermal nevus which follows the
Blaschko’s lines. Various treatment modalities including topical
agents, dermabrasion, cryotherapy, laser therapy, and partial-
thickness excision has been tried. Unfortunately, no one therapy
has been successful consistently. Giant ILVEN is a relative
contraindication to surgical excision and recurrence may be a
problem.

Inflammatory linear verrucous epidermal nevus (ILVEN) is an

epidermal nevus presenting with pruritus, erythema, and scaling
that occur as linear bands along the lines of Blaschko. Patients having
giant ILVEN seek treatment due to cosmetic disfigurement. Reported
therapeutic approaches include topical agents, dermabrasion,
cryotherapy, laser therapy, and partial-thickness excision.
Unfortunately, no one therapy has been successful consistently.[1]
Giant ILVEN is a relative contraindication to surgical excision and
recurrence may be a problem. We report the effectiveness of full
thickness excision and skin grafting for the definitive treatment of a
giant ILVEN.

Case Report
A 17 year old female presented with a linear pruritic lesion extending
from lateral border of right foot to right gluteal region [Figure 1]. The
parents noticed that lesion in the second month of life, which
continued to grow up to puberty. Since last 2 years it was stable.
Pruritus was intense leading to recurrent secondary infection. There
were no other systemic abnormalities. None of the family members
had similar type of lesion. She was prescribed oral, topical and
intralesional steroid, isotretinoin and methotrexate in past, without
any improvement. The routine investigations including tests for HIV
were within normal limits.
 Figure 1

Linear pruritic lesion extending from lateral border of right foot to right gluteal region at
presentation

Histopathology showed hyperkeratosis with alternate parakeratosis

and orthokeratosis and hypergranulosis beneath the orthokeratotic
area. There was mild spongiosis with psoriasiform epidermal
hyperplasia and mild to moderate perivascular lymphocytic infiltrate
in superficial dermis [Figure 2].
 Figure 2

Photomicrograph showing hyperkeratosis with alternate parakeratosis and orthokeratosis

and hypergranulosis beneath the orthokeratotic area (H and E, ×10)

A diagnosis of ILVEN was made on the basis of intense pruritus,

histopathological findings and lack of response to antipsoriatic
treatments. Considering the marriageable age, posteriorly placed
lesion and malignant potential, she had underwent full thickness
excision followed by split thickness skin grafting. The postoperative
period was uneventful. She was followed up for one year without any
recurrence or significant scarring [Figure 3]. The cosmetic result was
acceptable and the patient was satisfied.
 Figure 3

Follow up photograph of the patient after 3 months

Discussion
ILVEN is probably due to unidentified lethal dominant mutation,
rescued by mosaicism.[2] It is usually sporadic, but there have been
reports of familial cases.[3,4] Absent involucrin expression in the
parakeratotic epidermis has been reported, a finding that appears to
distinguish ILVEN from psoriasis.[5] About 75% appear during the
first five years of life, most often in the first six months, although
later onset has been recorded. ILVEN can be of any length,
occasionally extending the whole length of a limb. Usually it is
unilateral but bilateral and widespread lesions have been described.
Although generally persistent and resistant to treatment, some
lesions have been reported to resolve spontaneously.[6] Lichen
striatus, linear Darier disease, linear porokeratosis, linear lichen
planus, linear psoriasis, and the verrucous stage of incontinentia
pigmenti may all have similar clinical presentations as the linear
verrucous epidermal nevus. ILVEN can be distinguished from true
nevoid psoriasis by pruritus and lack of response to antipsoriatic
treatments. Lichen striatus is distinguished by its rapid development,
generally after the first year of life, by its relative lack of pruritus, by
its more lichenoid clinical and histological features, and, eventually,
by its spontaneous involution. The distinction between CHILD
syndrome and ILVEN is uncertain.

Potent topical corticosteroids applied under occlusion, or

intralesional steroid injections, provide little more than temporary
symptomatic relief.[7] There are few reports of effective systemic
treatments with acitretin and etanercept.[8,9] Laser ablation,
electrofulguration, cryotherapy, and medium to full-depth chemical
peels are other alternatives for smaller lesions. Earlier reports say,
surgical excision tends to be followed by rapid recurrence unless a
generous depth of underlying dermis is removed.[1,10] Lee et al.
reported 4 patients with extensive ILVEN treated successfully with
full-thickness surgical excision.

Our patient had an extensive lesion extending from right foot to

gluteal region and didn’t respond to any form of medical treatment.
The cosmetic results after full thickness excision and skin grafting in
our case was excellent without any recurrence within one year of
follow up. The patient was satisfied with the cosmetic outcome.

In conclusion, full thickness excision and skin grafting is a good

therapeutic alternative for extensive ILVENs and those not
responding to medical treatments.

What is new?

Our patient had an extensive lesion extending from right foot to

gluteal region and didn’t respond to any form of medical
treatment. The cosmetic results after full thickness excision and
 skin grafting in our case was excellent without any recurrence
within one year of follow up. The patient was satisfied with the
cosmetic outcome.

Footnotes
Source of Support: Nil
Conflict of Interest: Nil.

References
1. 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. [PubMed]
[Google Scholar]

2. 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. [PubMed] [Google Scholar]

3. Hamm H, Happle R. Inflammatory linear verrucous epidermal

nevus (ILVEN) in a mother and her daughter. Am J Med Genet.
1986;24:685–90. [PubMed] [Google Scholar]

4. Goldman K, Don PC. Adult onset of inflammatory linear epidermal

nevus in a mother and her daughter. Dermatology. 1994;189:170–2.
[PubMed] [Google Scholar]

5. Ito M, Shimuzu N, Fujiwara H, Maruyama T, Tezuka M.

Histopathogenesis of inflammatory linear verrucous epidermal
naevus. Arch Dermatol Res. 1991;283:491–9. [PubMed]
[Google Scholar]

6. Hodge SJ, Barr JM, Owen LG. Inflammatory linear verrucose

epidermal nevus. Arch Dermatol. 1978;114:436–8. [PubMed]
[Google Scholar]

7. Morag C, Metzker A. Inflammatory linear epidermal nevus: Report

of seven new cases and review of the literature. Pediatr Dermatol.
1985;3:15–8. [PubMed] [Google Scholar]
8. Renner R, Rytter M, Sticherling M. Acitretin treatment of a
systematized inflamatory linear verrucous epidermal naevus. Acta
Derm Venereol. 2005;85:348–50. [PubMed] [Google Scholar]

9. Bogle MA, Sobell JM, Dover JS. Successful treatment of a

widespread inflammatory verrucous epidermal nevus with
etanercept. Arch Dermatol. 2006;142:401–2. [PubMed] [Google Scholar
]

10. Landwehr AJ, Starinck TM. Inflammatory linear verrucous

epidermal nevus. Dermatologica. 1983;166:107–9. [PubMed]
[Google Scholar]

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