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CASE REPORT
Year : 2021  |  Volume : 12  |  Issue : 2  |  Page : 302-306  

Nevus unius lateris with bilateral oral mucosal lesions: An unusual presentation


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

Date of Submission17-Jun-2020
Date of Decision26-Jun-2020
Date of Acceptance13-Sep-2020
Date of Web Publication22-Feb-2021

Correspondence Address:
Lovleen Kaur
Senior Resident, Department of Dermatology, Venereology and Leprosy, Pt. B.D. Sharma Post Graduate Institute of Medical Sciences, Rohtak - 124 001, Haryana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/idoj.IDOJ_454_20

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   Abstract 


Verrucous epidermal nevi (VEN) are cutaneous hamartomas characterized by keratinocytic hyperplasia. Majority are linear in distribution and tend to follow the Blaschko lines; however, some may have zosteriform (segmental) or systematized distribution involving widespread areas of skin. The systematized ones are further classified into “Nevus Unius Lateris” when one-half of the body is affected, and “Ichthyosis Hystrix” showing bilateral distribution, both being the uncommon forms. Although it can affect any body part, it rarely involves the head and neck region with seldom involvement of mucosae, scalp, and ear lobes. We saw a 6-year-old child with multiple hyperpigmented verrucous plaques predominantly present over left half of the body, ipsilateral alopecia scalp, and verrucous lesions involving mucosae of palate and tongue, which were present bilaterally. Previously, case reports of oral lesions related to VEN had demonstrated segmental, midline, or unilateral distribution. Hereby, we report this peculiar case of Nevus Unius Lateris with bilateral oral mucosal involvement, owing to its rarity.

Keywords: Cutaneous mosaicism, nevus unius lateris, oral epidermal nevus, verrucous epidermal nevus


How to cite this article:
Kaur L, Mahajan BB, Mahajan M, Dhillon SS. Nevus unius lateris with bilateral oral mucosal lesions: An unusual presentation. Indian Dermatol Online J 2021;12:302-6

How to cite this URL:
Kaur L, Mahajan BB, Mahajan M, Dhillon SS. Nevus unius lateris with bilateral oral mucosal lesions: An unusual presentation. Indian Dermatol Online J [serial online] 2021 [cited 2021 Apr 18];12:302-6. Available from: https://www.idoj.in/text.asp?2021/12/2/302/308914




   Introduction Top


Verrucous epidermal nevi (VEN) are cutaneous hamartomas which originate from the embryonic ectoderm. The onset is usually at birth or early life with a prevalence of 1:1000 live births.[1] They are characterized by skin-colored to brown-gray papules which may coalesce to form plaques arranged in linear-streaks and swirls which do not generally cross the midline.[2] Presentation may be localized or widespread with different patterns such as linear, zosteriform and rarely, diffuse or systematized. When it involves extensive body surface area, the term “Systematized Epidermal Nevus” is preferred. Its variants include “Nevus Unius Lateris” when it covers one-half of the body and “Ichthyosis Hystrix” when distributed bilaterally.[3]


   Case Report Top


A 6-year-old female presented to Dermatology Outpatient Department with cosmetically disfiguring asymptomatic lesions in form of hyperpigmented verrucous plaques predominantly covering the left half of the body extending from scalp, face, external ear, neck, trunk, labium majus, left upper and lower limbs till the dorsum of left foot [Figure 1]a,[Figure 1]b,[Figure 1]c. Few thin streaks were also present over contralateral side of mid-face (lower eyelids, cheeks, upper lips), neck, shoulder [Figure 2]a and [Figure 2]b, and upper limb. The onset of the lesions occurred during infancy, and since then the lesions became darker, raised, and thickened with loss of hair over ipsilateral scalp. No history suggestive of developmental/milestones delay, seizures, mental retardation, and skeletal abnormality was present. Similar history in family was not present.
Figure 1: “Nevus Unius Lateris” on (a) anterior trunk with sharp midline demarcation and predominant left upper-limb involvement; (b) Posterior trunk. Note lesions over nape of neck and left-axilla are more verrucous; (c) Left lower limb involvement

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Figure 2: (a) Predominant left side involvement of face with few streaks involving contralateral skin of lower-eyelid, cheek, and upper-lip; (b) Left upper-eyelid involvement; Note paucity of hairs on left eyebrow

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On examination, the hairs over the left half of scalp were thin, hypopigmented, shorter in length, and sparse in distribution characteristically along the distribution of nevus with rest of the scalp having well-developed terminal hairs [Figure 3]a,[Figure 3]b,[Figure 3]c. Lesions over flexural surfaces such as right axilla, neck, cubital fossa and knuckles were more corrugated than other sites. On palpation, the lesions were soft and velvety to touch. Knuckles and volar aspect of left hand and dorsum of left great toe had multiple, thick, hyperkeratotic plaques [Figure 4]a,[Figure 4]b,[Figure 4]c. Oral examination revealed gray-colored tiny papules arranged in linear clusters over bilateral superior vermilion, left oral commissure, and adjoining buccal mucosa. Intraorally, papilliform mucosal plaques with irregular surface and borders involved midline and bilateral palatal mucosa, and dorsum of tongue [Figure 5]a,[Figure 5]b,[Figure 5]c,[Figure 5]d.
Figure 3: (a) Sparsely distributed hairs on left half of scalp with terminal hairs on contralateral side (posterior view); (b) Top view; (c) Lateral view with external ear involvement evident

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Figure 4: Thick hyperkeratotic lesions involving (a) thenar eminence, palmar surfaces of left thumb, and base of index finger; (b) PIP joints of left 4th and 5th fingers; (c) Dorsum of base of left great toe

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Figure 5: (a) Verrucous mucosal plaques on palate and dorsum of tongue; (b) Tongue lesions under magnification giving a cobblestone appearance; (c) Palatal mucosa under magnification; (d) Lip lesions. Note moustache-like pattern on upper lips with the extension of lesions from philtrum and philtral ridges to lip tubercle and both sides of superior vermilion respectively

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No systemic abnormality was detected. Routine investigations including electrocardiogram, ophthalmological examination, and Ultrasound abdomen were normal. Consent for CT-scan Brain was not given. Histopathology from upper back revealed massive hyperkeratosis, acanthosis, papillomatosis, and elongation of rete ridges. Increase in basal melanin was also evident [Figure 6]a and [Figure 6]b. Hence, the diagnosis of Nevus Unius Lateris with bilateral oral mucosal-VEN and ipsilateral alopecia of scalp was made. Though alopecia was remarkable along the distribution of nevus, no signs of scarring were evident.
Figure 6: (a) Presence of extensive hyperkeratosis, irregular acanthosis, papillomatosis, and elongation of rete ridges (H and E, 100×); (b) High basal melanin content observed at higher magnification (H and E, 400×)

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   Discussion Top


VEN represent a form of cutaneous mosaicism which emerges as a result of de-novo postzygotic mutations.[4] The common sites include trunk and extremities where lesions attain transverse and longitudinal configurations, respectively, following the Blaschko lines. It is uncommon over the head and neck region; rarely involves face, scalp, ear lobes[2] and very rarely affects the oral mucosa.[5] In our case, apart from the involvement of these sites, oral lesions were also recorded.

Historically, Brown and Gorlin in 1960 gave the first review on oral manifestations in verrucous epidermal nevus. Since then, only 16 cases of oral-VEN with or without cutaneous epidermal nevus have been described in the worldwide literature over a period of 60 years [Table 1]. Out of these, only a single case reported by Bygum et al. had systematized distribution of nevus (Ichthyosis Hystrix),[4] while nine cases had localized cutaneous involvement and six patients had isolated oral-VEN without any cutaneous feature.[6],[9],[10],[11] None of these patients had nervous, ocular or skeletal abnormalities, hence, did not fulfil the criteria of Epidermal Nevus Syndrome (ENS). On the other hand, there are multiple case reports in literature describing the oral manifestations in ENS. Worthy of note is the case of ENS described by Kelley et al. (1972) of a young boy having epidermal nevus involving the head and neck region. The boy also had blindness, mental retardation, and alopecia of scalp along the distribution of nevus. The oral lesions were highly disfiguring and mental deficiency led to factitious oral ulceration in this case.[12]
Table 1: Data of clinical features of oral-VEN observed by different authors

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Oral-VEN can involve any oral structure including lip vermilions, oral commissures, labial and buccal mucosa, gingivolabial sulcus, palatopharyngeal folds, hard or soft palate but they usually remain homo-lateral to their cutaneous counterparts. Their color may vary from normal-mucosa-like to yellow-white, tan, dark-brown, or gray. They usually present as unilateral/midline papules or sessile nodules with a verrucous, papillary, mammilated or condylomatous surface which characteristically do not cross the midline.[5],[6] However, the case reported by Bygum et al. of bilaterally systematized epidermal nevus had oral lesions which were also bilateral.[4] Although our case was predominantly Nevus Unius Lateris, the bilateral oral lesions could be explained by their bilateral cutaneous counterparts over mid-face (specifically cheeks and upper lips). These topographical considerations may imply that lines of Blaschko, originally illustrated in skin, may have intraoral counterparts caused due to proliferation of oral clones of epithelial progenitor cells.[9]

Histopathologically, oral-VEN shows papillomatous projections with a moderate degree of hyperkeratosis, acanthosis, and elongation of rete ridges which correlates clinically to the raised papillary lesions with a micropapillary or verrucous surface. The clinical diagnosis of oral-VEN is often supported by the presence of cutaneous lesions of VEN. However, the establishment of diagnosis becomes particularly difficult in the absence of cutaneous counterpart and differentiation from other oral diseases such as squamous papilloma, oral-verruca vulgaris becomes vital. Oral-VEN appears usually at birth or childhood, while squamous papilloma develops during 30–60 years of age. Also, squamous papilloma is usually small, pedunculated, and shows signs of keratinization with white surface, whereas oral-VEN is generally sessile, with the same color as normal mucosa.[6] Oral verruca vulgaris in children may clinically resemble oral-VEN; to differentiate, the former are acquired; sessile lesions and enlarge rapidly in a short timeframe with stabilization to a maximum size of 4–5 mm, whereas the lesions in latter have the tendency to constantly enlarge until their phase of somatic growth is over. Histopathologically, verrucae show finger-like projections of hyperkeratotic stratified-squamous epithelium producing a “cupping effect” with or without koilocytes.[9] In adults, it needs to be differentiated from verrucous carcinoma. The clinical correlation and history of onset and progression facilitate in ascertaining the correct diagnosis.

Treatment of oral-VEN lesions is generally not required if it does not cause any functional abnormality.[7] Fortunately, oral lesions in our patient were not problematic; in fact, they remained unnoticed until our examination. Surgical excision of oral lesions has been tried but recurrence was noticed as they tend to regrow during their phase of somatic growth till adolescence.[11] Considering possible malignant transformation which is well-documented in cutaneous VEN, no case of neoplastic transformation in oral-VEN is reported till date.[7] Nevertheless, systematized-VEN are managed symptomatically with appropriate counselling for the significance of long-term follow-up as the risk of malignant transformation, though rare, cannot be ruled out otherwise.[1]

Oral mucosal and scalp biopsy could not be performed in our case as parents denied the consent. This factor remains as a limitation. However, this case of Nevus Unius Lateris with alopecia scalp, genital and mucosal VEN in this young Indian girl is reported for its unusual combination of predominant unilateral cutaneous presentation and bilateral oral mucosal involvement.

Declaration of patient

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

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
De D, Kanwar AJ, Radotra BD. Basal cell carcinoma developing in verrucous epidermal nevus. Indian J Dermatol Venereol Leprol 2007;73:127-8.  Back to cited text no. 1
[PUBMED]  [Full text]  
2.
Thomas EA, Singla M, Shekhawat SS. Zosteriform verrucous epidermal nevus. Indian J Dermatol 2005;50:168-9.  Back to cited text no. 2
  [Full text]  
3.
Thomas VD, Snavely NR, Lee KK, Swanson NA. Benign epithelial tumors, hamartomas, and hyperplasias. In: Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, Wolff K, editors. Fitzpatrick's Dermatology in General Medicine. 8th ed. New York: McGraw-Hill; 2012. p. 1323-25.  Back to cited text no. 3
    
4.
Bygum A, Fagerberg CR, Clemmensen OJ, Fiebig B, Hafner C. Systemic epidermal nevus with involvement of the oral mucosa due to FGFR3 mutation. BMC Med Genet 2011;12:79.  Back to cited text no. 4
    
5.
Ozçelik D, Parlak AH, Oztürk A, Kavak A, Celikel N. Unilateral linear verrucous epidermal nevus of the face and the oral mucosa. Plast Reconstr Surg 2005;115:17e-9.  Back to cited text no. 5
    
6.
Haberland-Carrodeguas C, Allen CM, Lovas JG, Hicks J, Flaitz CM, Carlos R, et al. Review of linear epidermal nevus with oral mucosal involvement--series of five new cases. Oral Dis 2008;14:131-7.  Back to cited text no. 6
    
7.
Maly C, Olmo H, Stokes S, Cole H. Linear epidermal nevus: A case report with oral and facial manifestations. J Oral Maxillofac Surg 2016;74:2000-6.  Back to cited text no. 7
    
8.
Santos MD, Duarte AS, Carvalho GM, Guimarães AC, Zappelini CE, Coelho Dal Rio AC, et al. Linear epidermal nevus of the oral cavity: A rare diagnosis. Case Rep Med 2012;2012:206836.  Back to cited text no. 8
    
9.
Tesi D, Ficarra G. Oral linear epidermal nevus: A review of the literature and report of two new cases. Head Neck Pathol 2010;4:139-43.  Back to cited text no. 9
    
10.
Coley-Smith A, Shaw L. Oral findings in patients with epidermal naevi: A report of two cases. Dent Update 1996;23:158-60.  Back to cited text no. 10
    
11.
Hickman RE, Eveson JW, Cawson RA. Nevus unius lateris and intraoral verrucous nevi. Oral Surg Oral Med Oral Pathol 1988;66:226-9.  Back to cited text no. 11
    
12.
Kelley JE, Hibbard ED, Giansanti JS. Epidermal nevus syndrome. Report of a case with unusual oral manifestations. Oral Surg Oral Med Oral Pathol 1972;34:774-80.  Back to cited text no. 12
    


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