|LETTER TO EDITOR
|Year : 2015 | Volume
| Issue : 2 | Page : 46-48
Epidermolytic blaschkoid verrucous epidermal nevus: Report of two cases
Anupam Das, Indrashis Podder, Anirban Das, Anupama Ghosh, Kaushik Shome
Department of Dermatology, Medical College and Hospital, Kolkata, West Bengal, India
|Date of Web Publication||6-Jan-2016|
Department of Dermatology, Medical College and Hospital, Kolkata, West Bengal
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Das A, Podder I, Das A, Ghosh A, Shome K. Epidermolytic blaschkoid verrucous epidermal nevus: Report of two cases. Indian J Dermatopathol Diagn Dermatol 2015;2:46-8
|How to cite this URL:|
Das A, Podder I, Das A, Ghosh A, Shome K. Epidermolytic blaschkoid verrucous epidermal nevus: Report of two cases. Indian J Dermatopathol Diagn Dermatol [serial online] 2015 [cited 2019 Dec 16];2:46-8. Available from: http://www.ijdpdd.com/text.asp?2015/2/2/46/173418
Verrucous epidermal nevi (VEN) are congenital cutaneous hamartomas that occur as a result of excessive proliferation of the keratinocytes, reflecting genetic mosaicism arising from somatic mutation,  most commonly occurring in a Blaschko-linear distribution. Histologically, VEN shows hyperkeratosis, acanthosis, and papillomatosis. Based on the histology, it can be classified into two groups: Epidermolytic and non-epidermolytic.  Here, we report two cases of Blaschkoid VEN, which showed epidermolytic hyperkeratosis on histopathology.
A 16-year-old healthy girl presented with pruritic dark-colored elevated eruptions on the skin, which were present for the last 6 years. Cutaneous examination revealed numerous hyperpigmented warty papules coalescing into plaques, distributed in a patchy pattern roughly along the L3-L5, S2 dermatomes of the right lower limb. The lesions extended from the front of the knee to the dorsum of the foot (L3- L5), with a sharp cutoff at the medial border. Similar arrangement of lesions was also noted over the posterior aspect of the thigh (S2), extending to the heel. Some of the lesions sloughed off easily [Figure 1] and [Figure 2].
|Figure 1: Case 1 showing papules and plaques along the L4 and L5 dermatomes |
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|Figure 2: Case 1 showing papules coalescing to form plaques over the L3, L4, and L5 dermatomes (a) and close-up photograph demonstrating the verrucous surface of the lesions|
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Our second case was a 14-year-old healthy boy who presented with similar lesions of 8 years duration. Similar to the first case, these lesions were distributed along the L3, L5, and S1 dermatomes, affecting the right lower extremity. Cutaneous examination revealed warty papules and plaques in a Blaschkoid distribution over the front of the right knee (L3) extending to the dorsum of the foot (L5, S1) [Figure 3].
|Figure 3: Case 2 showing warty papules and plaques in a blaschkoid distribution over the front of the right knee (L3) extending to the dorsum of the foot (L5, S1)|
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Both the patients were born of non-consanguineous parentage, with normal birth and developmental history. The lesions were unrelated to seasonal variation. Past and family history were unremarkable. Hair, nails and mucosa were spared. Systemic review including skeletal, ophthalmic, and neurological survey was non-contributory. Routine laboratory investigations were within normal limits. Skin biopsy from representative lesions was done in both the cases. Histology showed hyperkeratosis, acanthosis, and papillomatosis. Perinuclear vacuolization of cells of stratum granulosum and stratum spinosum was present [Figure 4] and [Figure 5]. Based on the history, clinical findings, and histologic features, a diagnosis of "Blaschkoid verrucous epidermal nevus with epidermolytic hyperkeratosis" was made.
|Figure 4: Photomicrograph showing compact hyperkeratosis and vacuolization of the keratinocytes (H&E, 10×)|
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|Figure 5: Photomicrograph showing compact hyperkeratosis; perinuclear vacuolization of the keratinocytes in spinous and granular layers and increased number of basophilic keratohyalin granules in the stratum granulosum. (H&E, 40X)|
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Verrucous epidermal nevus (VEN) occurs as a result of hamartomatous proliferation of the keratinocytes.  It has been hypothesized that VEN is a mosaic disorder, resulting from post-zygotic mutations affecting the keratin 1 (K1) and/or keratin 10 (K10) gene(s).
Clinically, this disorder is characterized by verrucous skin and brown-colored discrete papules that often coalesce to form serpiginous plaques, affecting the extremities in most of the cases, along the Lines of Blaschko More Details. Rarely these lesions may be dermatomal in distribution. Bhagwat et al. reported a case where the lesions were distributed along the V1 dermatome (ophthalmic division of the trigeminal nerve),  while Thomas et al. reported zosteriform involvement of ear lobe.  Although most reports suggest a sporadic occurrence, few reports have documented familial occurrence; however, all such cases are inflammatory linear VEN (ILVEN). ,, Widespread VEN, especially involving the head and neck region, may be associated with the epidermal nevus syndrome.  Rarely, malignant transformation to basal and squamous cell carcinomas has been reported. 
Histologically, the hallmark finding of epidermal nevus is hyperkeratosis, acanthosis, and papillomatosis. Up to 62% of epidermal nevi show these features; the so-called nonepidermolytic epidermal nevi (NONEEN). Uncommon histopathologic varieties include epidermolytic hyperkeratosis (consistent with our case), acrokeratosis verruciformis, seborrheic keratosis, psoriasis (ILVEN), verruca vulgaris, porokeratosis,  focal acantholytic dyskeratosis etc.  Histological picture of epidermolytic hyperkeratosis is found in only about 16% of cases of epidermal nevi.  However, recently Meibodi et al. reported a case of ILVEN showing epidermolytic hyperkeratosis.  Koh et al. reported a case of systematized VEN with epidermolytic hyperkeratosis.  The main clinical differentials in our case included ILVEN, linear psoriasis, and lichen striatus. Absence of erythema and inflammation of the lesions ruled out ILVEN. Resistance to conventional treatment and severe pruritus helped to rule out linear psoriasis (linear nevoid psoriasis has mild pruritus). Absence of basal layer degeneration and band like lymphocytic infiltrate on histopathological examination helped to rule out lichen planus and lichen striatus (lichenoid disorders have basal cell degeneration, band like lymphocytic infiltration).
On histological grounds, epidermolytic hyperkeratosis (EH) is a minor pathological reaction pattern of skin, first described by Ackerman in 1970. This pattern has been observed in many congenital or acquired, benign and malignant skin lesions that have been shown in [Table 1]. In both cases, the patients were prescribed topical retinoids and were advised regular follow-up. To sum-up, EHK in VEN is a rare occurrence, thus, evoking the current report.
|Table 1: Dermatoses that may show epidermolytic hyperkeratosis on histopathology|
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]