Epidermodysplasia Verruciformis

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epidermodysplasia verruciformis (EV):

By: Maricel Zamoras October 24, 2012 epidermodysplasia verruciformis (EV)

DISEASE PROCESS:

DISEASE PROCESS Rare, autosomal recessive inherited skin disorder characterized by eruptions of wart-like lesions that may occur anywhere on the body Lesions are caused by infection with the Human Papillomavirus (HPV) O ver 70 subtypes of HPV that cause a wide range of viral warts Most people (with the infection of some subtypes of HPV) have minimal or no clinical effect, but people who have EV and an infection with these subtypes of HPV can have flat, wart-like lesions Clients with EV have an abnormal or impaired immune response to HPV or wart viruses

DISEASE PROCESS:

DISEASE PROCESS Wart-like lesions may transform into skin cancers Malignant skin tumors (carcinomas), especially squamous cell carcinoma (in situ or invasive), develop frequently Skin cancers initially appear on sun-exposed areas, such as the face, neck, chest, and arms Usually infected with multiple types of HPV (i.e. HPV type 3 and 10) More than 30 EV-HPVs (i.e. Types 4, 5a, 5b, 8, 9, 12, 14, 15, 17, 19-25, 36-38, 47, and 50) have been identified in EV lesions

RISK FACTORS :

RISK FACTORS Genetics, autosomal recessive Infections HIV and AIDS Organ Transplants Other conditions associated with long-term immunosuppression People with immunosuppression and a chronic infection of HPV may lead to formation of skin lesions and cancer

SIGNS AND SYMPTOMS :

SIGNS AND SYMPTOMS Symptoms are very diverse Skin lesions on exposed part of the body Face (primarily the forehead) Dorsal part of the hands Forearms or lower leg areas Symptoms do not apply to mucous membranes and the products of the epidermis (hair, nails) Skin lesions vary From simple and benign as erythema and hyperpicmentation to flat warts resembling lumps of different colors Flattened and warty skin lesions Flat red-brown macules Skin bumps on back of hand Scaly maculopapular lesions, resembling tree bark

REAL LIFE STORIES :

REAL LIFE STORIES Dede Koswara from Indonesia Known as “Tree Man” Has spent much of his life covered in tree-like warts Condition began when he was 15 and cut his knee in an accident Small wart developed on his lower leg and spread uncontrollably Koswara’s immune system has made him unable to fight the Human Papilloma Virus infection causing his body to produce warts/lesions http:// www.youtube.com / watch?v =Vtl9gN0gTvs

TREATMENT :

TREATMENT Medications acitretin interferons retinoids Synthetic Vitamin A Excision of lesions Surgery Recommended the skin lesions are regularly assessed and removed if malignancy develops Avoid the sun There is no definitively effective treatment

REAL LIFE STORIES :

REAL LIFE STORIES

AGE OF MANIFESTATION OF SYMPTOMS :

AGE OF MANIFESTATION OF SYMPTOMS Condition applies to all age groups including children but the largest proportion is in patients aged 25-58 years Age at onset is variable; more frequently: young adults or children

EVALUATING THE DISEASE PROCESS :

EVALUATING THE DISEASE PROCESS Biopsy is performed for early detection of premalignant and malignant lesions Most characteristic findings are seen within the epidermis Classic histological manifestation of EV is a verruca plana -like lesion with mild hyperkeratosis and acanthosis

GENETICS:

GENETICS Autosomal recessive disease Caused by an inactivating HP mutation in either the EVER1 or EVER2 genes Located adjacent to one another on Chromosome 17

REAL LIFE STORIES :

REAL LIFE STORIES An MD from the University of Maryland’s School of Medicine worked with local doctors and designed a drug treatment program The put him on a chemotherapy regimen but it was cut short because Koswara’s liver began to fail Administered synthetic vitamin A Local Indonesian surgeons also removed 13 pounds of warts and decaying matter

EXPECTED OUTCOMES :

EXPECTED OUTCOMES EV lesions and tumors evolve progressively Fatal occurrence in an African man developed squamous cell carcinoma of the conjunctiva He had ocular invasion causing blindness, metastasis, and eventually death http://www.ncbi.nlm.nih.gov/pubmed/ 14576647 Clients with EV generally have weak immune systems Moderate life expectancy if there is no other life threatening condition I.E. Cancer, TB, HIV AIDS, liver failure, etc.

NURSING IMPLICATIONS:

NURSING IMPLICATIONS Avoid cutting, shaving, brushing, or picking lesions Wear shower shoes in locker rooms or public showers Wash hands carefully after touching a lesion Keep hands and feet dry and clean as possible Greater chance of organisms growing in warm and moist environments Be sensitive to the mental/emotional damage Many clients become depressed/suicidal Prevent infection Adhere to treatment program Chemotheraphy , medicine, surgeries, etc.

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