Epidermodysplasia verruciformis

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Epidermodysplasia verruciformis, by Wikipedia https://en.wikipedia.org/wiki?curid=10024855 / CC BY SA 3.0

#Virus-related_cutaneous_conditions
#Rare_diseases
#Papillomavirus-associated_diseases
#Epidermal_nevi,_neoplasms,_and_cysts
#Defects_in_innate_immunity
Epidermodysplasia verruciformis (EV), also known as treeman syndrome, is an extremely rare autosomal recessive hereditary skin disorder associated with a high risk of skin cancer.
It is characterized by abnormal susceptibility to human papillomaviruses (HPVs) of the skin.
The resulting uncontrolled HPV infections result in the growth of scaly macules and papules resembling tree bark, particularly on the hands and feet.
It is typically associated with HPV types 5 and 8, which are found in about 80% of the normal population as asymptomatic infections, although other types contribute less frequently,
among them types 12, 14, 15 and 17 (which are occasionally referred to as the beta papillomaviruses).
The condition usually has an onset of between the ages of one and 20 but it can occasionally be present in middle age.
The condition is also known as Lewandowsky–Lutz dysplasia, named after the physicians who first documented it, Felix Lewandowsky and Wilhelm Lutz.
Clinical diagnostic features are lifelong eruptions of pityriasis versicolor-like macules, flat wart-like papules, one to many cutaneous horn-like lesions, and development of cutaneous carcinomas.
Patients present with flat, slightly scaly, red-brown macules on the face, neck, and body, recurring especially around the penial area, or verruca-like papillomatous lesions,
seborrheic keratosis-like lesions, and pinkish-red plane papules on the hands, upper and lower extremities, and face.
The initial form of EV presents with only flat, wart-like lesions over the body, whereas the malignant form shows a higher rate of polymorphic skin lesions and development of multiple cutaneous tumors.
Generally, cutaneous lesions are spread over the body, but some cases have ...




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Tags:
Defects in innate immunity
Rare diseases