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Pathology of Epidermal

Nevus and Inflammatory

Linear Verrucous

Epidermal Nevus (ILVEN)

"Developmental malformations of the epidermis"

Dr Sampurna Roy MD     


Dermatopathology Quiz Case 165

Diagnosis: Inflammatory Linear Verrucous Epidermal Nevus




Epidermal Nevus: 

Epidermal nevus is a developmental malformation of the epidermis. 

These lesions are commonly located on the neck, trunk or extremities.

Macroscopically epidermal nevi present as warty brown plaque, scaly  discolouration or linear lesions.

Microscopically different patterns are noted:

The common variant displays laminated hyperkeratosis, papillomatosis  and acanthosis.

There is thickening of the granular layer and mild increase in basal melanin pigment.

Some of the rare histologic patterns resemble seborrheic keratosis , acrokeratosis verruciformis or epidermolytic hyperkeratosis.  

Inflammatory Linear Verrucous Epidermal Nevus:  (ILVEN)

Inflammatory Linear Verrucous Epidermal Nevus, also known as ILVEN, is clinicopathological subgroup of epidermal nevus.

It resembles linear psoriasis both clinically and histologically.

The lesion presents as persistent linear, pruritic eruptions.

It is composed of erythematous, slightly verrucous scaling papules arranged in one or several lines on the lower extremities.

The usual time of onset is early childhood.

The disease may occur in adults.

The histologic feature is usually that of a non-specific chronic dermatitis.

There is psoriasiform epidermal hyperplasia and overlying alternate parakeratosis and orthokeratotic areas of hyperkeratosis.

Important microscopic features:   Histopathology images

- Hyperkeratosis, moderate acanthosis with elongation of rete ridges

- There is mild spongiosis.

- Characteristic histologic feature is regular alternation of slightly raised parakeratotic areas without a granular layer (hypogranulosis) and slightly depressed orthokeratotic areas with prominent granular layer (hypergranulosis).

- The orthokeratotic area shows a basket-weave-pattern.

- The dermis shows scattering of chronic inflammatory infiltrate.

Note: Orthokeratotic hyperkeratosis is characterised by hyperkeratosis with non-nucleated cells

Parakeratotic hyperkeratosis is characterised by hyperkeratosis with nucleated cells.


Further reading:

(ILVEN) and psoriasis.

Epidermal nevus syndrome.

Verrucous epidermal nevus.

Epidermal nevus syndrome: epithelial and cutaneous tumours without systemic disorders: a case report.

Analysis of mutations in the PIK3CA and FGFR3 genes in verrucous epidermal nevus.

Immunohistochemical differentiation between inflammatory linear verrucous epidermal nevus (ILVEN) and psoriasis.

Epidermal nevus syndromes: clinical findings in 35 patients.

Mycosis fungoides mimicking inflammatory linear verrucous epidermal nevus.

Inflammatory linear verrucous epidermal nevus.

Acantholytic dyskeratotic epidermal nevus: a rare histopathologic feature.

Adult onset verrucous epidermal nevus.

The spectrum of epidermal nevi: a case of verrucous epidermal nevus contiguous with nevus sebaceus.

Bilateral inflammatory linear verrucous epidermal nevus associated with psoriasis.




Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


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