Epidermodysplasia Verruciformis was first reported by Lewandowsky and Lutz in 1922, characterized by generalized flat wart-like skin lesions throughout the body. It is also known as verrucous epidermal dysplasia.

bubble_chart Etiology

It was previously considered a genetic disease, but this condition can be autoinoculated and heteroinoculated. Electron microscopy has revealed papillomavirus particles within the intranuclear inclusions of affected cells, confirming that this condition is a generalized verruca. To date, six types of HPV have been isolated from the lesions, including types 3, 5, 8, 9, 10, and 12, with HPV-3 and HPV-5 being the predominant types. HPV-5 has potential carcinogenic properties. However, malignant transformation of the lesions is observed only in sun-exposed areas, leading some to suggest that sunlight injury is the primary factor in malignant transformation, while the wart virus may merely be an additional factor, possibly interacting with the patient's unique constitution.

bubble_chart Clinical Manifestations

The condition often begins in childhood but can occur at any age. The rash consists of flat, wart-like papules ranging from the size of a grain of rice to a soybean, round or polygonal in shape, and firm in texture. They appear pale gray, dark red, purplish-red, or brown. The number of lesions gradually increases and is symmetrically distributed. They commonly affect the face, neck, trunk, and limbs, but may also spread across the entire body, with small wart-like lesions even appearing on the lips and urethral orifice. The rash is most numerous and densely clustered on the face, neck, and back of the hands, resembling flat warts. On the trunk, the lesions are larger and harder, similar to common warts. Additionally, it is often accompanied by metatarsal keratosis, nail deformities, freckle-like nevi, and delayed intellectual development. Some patients may experience cutaneous pruritus. The disease progresses very slowly, persisting for years without resolution. Approximately 20% of patients may develop squamous cell carcinoma or basal cell carcinoma from the skin lesions.

Histopathology: Hyperkeratosis of the epidermis, acanthosis, vacuolization of cells in the granular and spinous layers forming a basket-weave pattern. Although the cytoplasm is completely dissolved and vacuolated, the nuclei remain intact, with no dyskeratotic cells observed.

bubble_chart Diagnosis

The diagnosis can be made based on generalized flat wart-like rashes and histopathological changes.

bubble_chart Treatment Measures

No satisfactory treatment is available. Often, there are too many lesions to treat, so only the troublesome ones are removed. Freezing, electrocautery, and X-ray therapy can be used.

bubble_chart Differentiation

(1) Acrokeratosis verruciformis: The rash commonly occurs on the back of the hands and feet, knees, elbows, etc. It presents as flat, wart-like papules. Histopathology: No vacuolation is observed in the upper epidermal cells.

(2) Lichen planus: Characterized by purplish-red papules with intense itching. Mucous membrane lesions are often present. Pathology: Hyperkeratosis, irregular acanthosis, liquefaction degeneration of basal cells, and band-like lymphocytic infiltration in the dermis.