|Year : 2015 | Volume
| Issue : 4 | Page : 239-242
Verrucous epidermal nevus with cicatricial alopecia over scalp
Nilofar G Diwan, Abhishek P Pilani, Pragya A Nair
Department of Dermatology, Pramukhswami Medical College, Karamsad, Anand, Gujarat, India
|Date of Web Publication||24-Sep-2015|
Pragya A Nair
Department of Dermatology, Pramukhswami Medical College, Karamsad, Anand, Gujarat
Source of Support: None, Conflict of Interest: None
Verrucous epidermal nevus (VEN) is a common type of keratinocyte hamartoma present at birth or occurring later in life. It is seen at any site, but is less common on the head and neck, rarely seen on the face and very rarely involves the oral mucosa. Widespread multiple epidermal nevi may reflect genetic or chromosomal mosaicism. Blaschko has documented characteristic linear, zosteriform, unilateral or systematized patterns with streaks and swirls. They are resistant to treatment with risk of recurrence. A case of male child is presented here with extensive VEN with involvement of ear lobe and scalp of left side with cicatricial alopecia. Patient had delayed milestone development without any systemic involvement.
Keywords: Blaschko′s lines, cicatricial alopecia, verrucous epidermal nevus
|How to cite this article:|
Diwan NG, Pilani AP, Nair PA. Verrucous epidermal nevus with cicatricial alopecia over scalp. Indian J Paediatr Dermatol 2015;16:239-42
|How to cite this URL:|
Diwan NG, Pilani AP, Nair PA. Verrucous epidermal nevus with cicatricial alopecia over scalp. Indian J Paediatr Dermatol [serial online] 2015 [cited 2019 Jul 18];16:239-42. Available from: http://www.ijpd.in/text.asp?2015/16/4/239/165634
| Introduction|| |
Epidermal nevi are hamartomas of the skin and have multiple clinical variants, including a verrucous type.  Epidermal nevi occurring on the head and neck are likely to be sebaceous nevus, while those occurring elsewhere are more likely to be verrucous epidermal nevus (VEN). 
Verrucous epidermal nevus is a common type of keratinocyte hamartoma present at birth or occurring later in life. They affect about one in every 1000 live births. It is seen as vertical, linear or s-shaped lesion, and does not normally cross the midline. 
Linear VEN (also known as a "linear epidermal nevus," or "VEN") is a skin lesion characterized by a verrucous skin-colored, dirty-gray or brown papules.  When this nevus covers a diffuse or extensive portion of the body's surface area, it may be referred to as a systematized epidermal nevus, when it involved only one-half of the body it is called a nevus unius lateris. 
It is seen at any site but is less common on the head and neck, rarely seen on the face and very rarely involve the oral mucosa.  They are resistant to treatment with risk of recurrence. Here we report a case of an 8-year-old boy with linear VEN over body mainly over left side with cicatricial alopecia of scalp on the same side.
| Case report|| |
A male child aged 2 years presented to skin outpatient department with a complaint of multiple lesions all over the body since birth and cough, cold, and fever since almost 15 days. History of dropping of saliva from left angle of mouth and dropping of left eyelid since 3-4 days was also present in 2008. There was no history of refusal of feeds, convulsions, excessive cry or vomiting. Family h/o of nevus in father and sister present with no consanguinity in parents,
Cutaneous examination showed verrucous hyperpigmented papules and plaques over left side of body particularly over arm, inner aspect of forearm, medial aspect of thigh, inner aspect of lower leg, dorsum of hand and feet. Verrucous hyperpigmented plaques were also present over nape of neck, front of neck, left side of the face, left eyelid, and left retroauricular area. On chest, back and abdomen, multiple hyperpigmented papules and plaque were along the lines of Blaschko's [Figure 1]. Callosity-like lesions were present over left elbow and hand [Figure 2]. Hyperpigmented papules were present over right side also mainly over the inner arm, chest, face, abdomen, and axilla [Figure 1].
|Figure 1: Multiple hyperpimented papules and plaque along the lines of blaschkos over left chest, back and abdomen and also over right side|
Click here to view
Hyperpigmented papules were present over forehead with no hair follicles over left frontal, temporal, and parietal region on left side of scalp [Figure 3]. Drooping of left eyelid with deviation of angle of mouth was present.
|Figure 3: Hyperpigmented papules over forehead without hairfollicles over left frontal, temporal and parietal region of scalp|
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Computed tomography (CT) scan was done which was normal. Relatives disagreed to give consent for biopsy. Patient was given symptomatic treatment and counseled to come for follow-up regularly. After 6 years in 2014, the patient presented with increased verrucous plaques with infection in axilla [Figure 4], verrucous plaques over neck and left side of face [Figure 5], cicatricial alopecia over scalp [Figure 6], and drooping of left eyelid [Figure 7]. Due to financial constraints CT scan or MRI was not done. Milestones of the patient were delayed with no other systemic symptoms.
Patient was clinically diagnosed as a case of VEN along the line of blaschko's with cicatricial alopecia over left side of the scalp.
| Discussion|| |
Epidermal nevi are hamartomatous lesions arising from the embryonic ectoderm. VEN are congenital, noninflammatory cutaneous hamartomas composed of keratinocytes, each lesion comprising the progeny of a single mutant keratinocyte. 
Linear VEN are linear hamartomas of epidermal structures that usually appear at birth or during infancy. Clinically, there is no erythema or pruritus. Lesions are flat, velvety, papillomatous in the newborn have been distinguished as "soft," whereas, more keratotic, verruciform lesions during adolescence are described as "hard," may vary from skin colored to brown. 
Epidermal nevi most commonly present as a single linear lesion, but sometimes multiple unilateral or bilateral linear plaques are seen. Most lesions consist of well-circumscribed, hyperpigmented, papillomatous papules or plaques that are usually asymptomatic. Rarely, epidermal nevi are hypopigmented. Once developed, the nevi may thicken and become more verrucous, especially over joints and in flexural areas such as the neck as seen in our case.
Verrucous epidermal nevus occurs in circumscribed patches or more often, in linear streaks or whorls following Blaschko's lines,  suggesting that they represent postzygotic mutations. Blaschko documented that linear streaks and swirls adopted by these nevi may be due to somatic mutation, comprising the progeny of a single mutant keratinocyte. Widespread multiple epidermal nevi may reflect genetic or chromosomal mosaicism. Blaschko has documented characteristic linear, zosteriform, unilateral or systematized patterns with streaks and swirls.  In some cases, when there are multiple lesions, there may be associated defects in other tissues, particularly the skeleton and the central nervous system, then called "epidermal nevus syndrome." 
The common sites are trunk and limbs and involvement of the head and neck region and involvement of the ear lobe is rarely reported , as in our case where involvement of ear and scalp was seen with cicatricial alopecia.
A case of linear tan colored verrucous skin lesions over right side of the face, extending to the scalp, right upper and lower limbs is reported where hair were long, fuzzy, differing in texture with patchy alopecia. Verrucosity of the lesions and the extensive distribution suggested it as VEN. As histopathological examination could not be done from the lesion over the scalp, it was difficult to differentiate it from nevus sebaceous, same as in our case. 
Risk of developing basal cell carcinoma in a nevus sebaceous, a type of epidermal nevus is higher (6.550%), compared to the more common VEN, in spite of the fact that both arise from pluripotent primary epithelial germ cells which have the capacity to differentiate different epithelial structures. 
Histologically, VEN are characterized by acanthosis, orthohyperkeratosis, papillomatosis, and an expanded papillary dermis sharply demarcated from the surrounding normal skin.  Immunohistochemical studies further help differentiate inflammatory linear (IL) VEN from other non-IL epidermal nevus.
The treatment of VEN is difficult and often unsatisfactory. Treatment involves topical therapy such as corticosteroids, retinoic acid, tars, anthralin, 5-fluorouracil, and podophyllin, but they are of limited benefit. Surgical modalities include excision with full thickness graft, cryotherapy,  carbon dioxide laser vaporization,  and erbium: YAG laser therapy. 
It is important to have a long term follow-up in such cases as there are chances of development of malignancy.
| Conclusion|| |
Case is presented for its rarity in the form of extensive VEN in a male gender with involvement of ear lobe and scalp of left side with cicatricial alopecia.
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Conflicts of Interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]