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Pathology of Epidermodysplasia Verruciformis

Dr Sampurna Roy MD            

 

                                                                                                                      

 

 

Syn: Lewandowsky-Lutz dysplasia

Epidermodysplasia verruciformis (EV) was initially described by Lewandowski and Lutz in 1922.

Epidermodysplasia verruciformis (EV) is a rare, inherited disorder in which there is widespread and persistent infection by multiple subtypes of human papilloma virus. The patients have high risk of cancer.

Epidermodysplasia Verruciformis usually occurs in infancy and childhood.

Clinically the lesion presents as flat or papular wart, scaly macule or hypopigmented tinea versicolor-like lesions.

These are usually located on the face, neck or upper trunk.

Numerous HPV subtypes are associated with Epidermodysplasia Verruciformis.

HPV 5 and 8 are known to have highest oncogenic potential.

Some lesions are similar to plane warts where as others resemble seborrheic keratosis.

Microscopic  features:

    

Meireles SIF do C, de Andrade SMF, de Lima Jśnior CLH, Pires MC. Epidermodysplasia Verruciformis in a young man with HIV since birth - Case report . Anais Brasileiros de Dermatologia. 2013;88(6 Suppl 1):190-192. doi:10.1590/abd1806-4841.20132328.

Mendes AD, Bittencourt M de JS, Moure ERD, D’Macźdo CM, Yamaki IN, de Araśjo DM. Acquired epidermodysplasia verruciformis in a renal transplant recipient - Case report . Anais Brasileiros de Dermatologia. 2014;89(1):144-146. doi:10.1590/abd1806-4841.20142803. 10.1590/abd1806-4841.20142803

- Histologically, the lesions display epidermal thickening with swollen cells in the upper epidermis.

- There are nests of large cells with prominent perinuclear halo and greyish blue cytoplasm.

- Keratohyaline granules of different shapes and sizes may be present within the swollen cells.

- Stratum corneum shows basket weave like pattern.

- Some epidermal cells show dysplastic changes.

Almost half of all patients with Epidermodysplasia verruciformis will develop cutaneous malignancies like Bowen's type carcinoma-in situ and invasive squamous cell carcinoma.

These lesions usually occur on sun exposed areas in the fourth of fifth decade of life. 

Read related posts:  Verruca vulgaris; Palmoplantar wart; Verruca plana ; Condyloma Acuminatum

 

Further reading:

Acquired Epidermodysplasia Verruciformis Due to Multiple and Unusual HPV Infection Among Vertically-Infected, HIV-Positive Adolescents in Zimbabwe

Human papillomavirus infection and UV exposure as epidermoid inclusion cyst risk factors in a patient with epidermodysplasia verruciformis?

Epidermodysplasia Verruciformis

Epidermodysplasia verruciformis. Clinical and light-and electron-microscopic observations during etretinate therapy

Genetics of Epidermodysplasia Verruciformis

Human papillomavirus and squamous cell cancer of the skin–epidermodysplasia verruciformis-associated human papillomavirus revisited

The histologic spectrum of epidermodysplasia verruciformis in transplant and AIDS patients.

Do epidermodysplasia verruciformis human papillomaviruses contribute to malignant and benign epidermal proliferations?

The histologic spectrum of epidermodysplasia verruciformis.

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

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