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Epidermodysplasia Verruciformis
Introduction
Epidermodysplasia verruciformis is a rare genetic dermatologic condition in which patients show a compromised immunologic ability to defend against and eradicate certain types of human papillomavirus (HPV). This leads to persistent infections and an increased lifetime risk of developing cutaneous dysplasia and malignancy. The connection between viral infections and certain forms of carcinogenesis, especially with HPV, is well established. Specific types of HPV are more strongly associated with the development of dysplasia and carcinogenesis.
Individuals with this condition develop HPV-derived cutaneous lesions at a significantly higher rate than the general population. This genodermatosis manifests mainly as verrucous cutaneous lesions such as multiple persistent verrucae, pityriasis versicolor-like lesions, and other verrucous or "warty" cutaneous lesions. In addition, this also leads to the development of Bowen disease and squamous cell carcinoma. The lesions associated with epidermodysplasia verruciform tend to exhibit a characteristic histopathologic appearance. Although epidermodysplasia verruciformis is a rare genetic disease, extensive research has provided insights into viral infection and its role in carcinogenesis pathways.[1] This condition exists in broadly 2 forms:
- The classic form is also the inherited or primary and follows an autosomal recessive pattern.
- The acquired form is the secondary type and is clinically almost indistinguishable. This is mainly observed in HIV-infected, immunocompromised, or immunosuppressed individuals.[2][3]
Etiology
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Etiology
Epidermodysplasia verruciformis is caused by a reduced ability of the immune system to fend off and eradicate HPV infection.[4] Since the condition was first described by Lewandowsky and Lutz in 1922, many different types of HPV have been implicated in the development of cutaneous lesions in affected patients.[5] The most commonly identified HPV types found in epidermodysplasia verruciformis–related cancers are HPV types 5 and 8, which are present in up to 90% of related skin cancers.[6] Other types of HPV that are alternatively found include 9, 12, 14, 15, 17, 19, 20, 21, 22, 23, 24, 25, 36, 38, 47, and 50.[7][8] A study showed that HPV 5 was the most common single type isolated, and contrary to other literature results, HPV types 3, 14, and 20 were more commonly isolated than HPV 8.[9]
This autosomal recessive condition, in its primary or inherited form, is caused by a mutation in the genes TMC6/EVER1 or TMC8/EVER2, which is believed to impart a defect in the ability to mount an immune response to certain HPV types within keratinocytes.[10] Patients with this typical form do not generally display reduced immune capabilities against other infectious pathogens. The beta-HPV types identified in affected patients who develop skin malignancies are also found in the general population and individuals without the EVER mutations or epidermodysplasia verruciformis. Notably, these HPV types have not been shown to produce dysplasia or malignancy otherwise.[11] Malignant transformation of cutaneous epidermal lesions in epidermodysplasia verruciformis is usually observed in association with HPV types 5, 8, 17, 20, and 47. These lesions usually develop in patients in their 30s, decades after the initial presentation.[12][13][14][5]
In patients with the acquired form of epidermodysplasia verruciformis, also known as the atypical form, there is usually a history of some form of immunodeficiency, often treatment-related. This occurrence is believed to be specifically due to T-cell–mediated immunodeficiency in patients with predisposing genetic mutations, such as RHOH and STK4. However, not all immunosuppressed patients with these mutations develop epidermodysplasia verruciformis–like disease. This differs from the typical form, wherein characteristic mutations are inherited and display full penetrance.[15][16]
Interestingly, a case has been reported of a patient who developed epidermodysplasia verruciformis lesions in an acquired fashion due to trauma from Brazilian hot wax hair removal of the pubic region. The patient had a history of renal transplant 5 years earlier and a pancreatic transplant 3 years earlier. The area was denuded from the hot wax treatment, and a brownish-red cutaneous plaque developed as it healed. A biopsy confirmed the lesion as epidermodysplasia verruciformis. The patient denied any family or personal history of epidermodysplasia verruciformis lesions. A portion of the lesion was successfully treated with topical 0.1% tretinoin, which resulted in better resolution and less post-inflammatory pigmentation compared to cryotherapy used concurrently on another portion of the lesion.[17]
Epidemiology
Epidermodysplasia verruciformis is an extremely rare condition. A literature review by Imahorn et al in 2017 identified approximately 500 reported cases of this condition worldwide.[9] Patients with typical epidermodysplasia verruciformis develop lesions early in childhood and continue to develop new lesions throughout their lives.[11] These cutaneous lesions persistently infected with beta-HPV may progress to non-melanoma skin cancer, with 30% to 70% of affected patients developing squamous cell carcinoma over their lifetime.[18][19][20] No gender or geographical bias has been identified.[21]
Pathophysiology
Most HPV infections within cutaneous keratinocytes are cleared in the general population without progression to malignancy. In the general population, HPV types 3 and 10 are primarily associated with verruca vulgaris.[2] However, individuals with epidermodysplasia verruciformis exhibit a diminished innate ability to clear HPV infections, resulting in persistent infection and progression to dysplasia and malignancy. This condition manifests as widespread pityriasis versicolor–like and verruca plana–like lesions, along with an increased risk of persistent beta-HPV infection, leading to the development of non-melanoma skin cancer.
Mutations in TMC6/EVER1 or TMC8/EVER2 genes account for over 50% of the mutations responsible for the inherited form of disease.[10] The exact cellular and molecular mechanisms that lead to increased susceptibility to HPV infection and persistence within keratinocytes are not fully understood. However, some hypotheses suggest that TMC6/EVER1 or TMC8/EVER2 function as transmembrane channel proteins that help limit viral replication and gene expression of beta-HPV within keratinocytes.[22] These proteins may also affect zinc transporters, thereby influencing intracellular zinc concentration. Zinc plays a crucial role in transcription factors that activate steps in the HPV life cycle, such as AP-1, which is involved in HPV cellular proliferation.[23] In addition, Merkel cell polyomavirus has been identified in epidermodysplasia verruciformis lesions.[24]
Histopathology
Histopathological examination of epidermodysplasia verruciformis lesions reveals various features of verruca-type lesions. Characteristic findings of epidermodysplasia verruciformis–type lesions include hyperkeratosis and parakeratosis, acanthosis with koilocytic cellular atypia, and pale eosinophilic cytoplasm. The histological findings depict "blue cells," which are pathognomonic for HPV-infected keratinocytes and can indicate epidermodysplasia verruciformis–related disease. The "blue" appearance refers to cells exhibiting pale blue cytoplasm and abundant basophilic keratohyalin granules.[25] Actinic keratoses and Bowen-type lesions may also be observed. Actinic keratoses appear disordered and atypical keratinocytes in the superficial epithelium but do not exhibit full-thickness epidermal atypia. Bowen-type lesions are characterized by a pagetoid distribution of highly atypical keratinocytes within the epidermis.
History and Physical
Patients with both inherited and acquired epidermodysplasia verruciformis present with similar lesions, characterized by verruca plana-like, sometimes scaly, often flat lesions, and papules, and are usually found on the trunk, neck, or face. These lesions can also resemble those of pityriasis versicolor lesions.[26] These lesions appear in multiple locations and, in the case of inherited epidermodysplasia verruciformis, they may begin as early as infancy and persist throughout the patient's life. They usually appear on skin areas exposed to UV light. A family history of epidermodysplasia verruciformis is helpful for diagnosis. For acquired epidermodysplasia verruciformis, a history of HIV or immunosuppression medical therapy is crucial for diagnosis.[11][5]
Evaluation
Definitive evaluation and diagnosis of epidermodysplasia verruciformis rely on a dermatologist's clinical examination and a pathologist's histopathological analysis of the suspected skin lesions. Indeed, while the diagnosis of epidermodysplasia verruciformis can be challenging due to its rarity and the potential lack of clinician awareness, the characteristic histopathological findings, especially when coupled with a family history or early development of multiple similar lesions, can aid in making the diagnosis. Dermatologic clinical evaluation and histopathologic examination of suspected lesions are essential for accurate diagnosis. Molecular analysis to identify known mutations associated with epidermodysplasia verruciformis is now available.[27][28]
Treatment / Management
A definitive treatment for epidermodysplasia verruciformis does not exist.[29] However, several possible treatments are recommended—from excision to other therapies such as acitretin, imiquimod, interferons with retinoids, topical calcipotriol, and cimetidine (although its efficacy toward the treatment is debated).[30] As the lesions tend to occur in sun-exposed areas of the skin, sun exposure protection counseling and adherence are crucial in treating patients with this condition. Patients with epidermodysplasia verruciformis require annual or more frequent checkups with dermatologists to monitor for the development of new, concerning lesions.[31][32][33][34] Any suspiciously malignant lesions should be excised to evaluate histopathologically. Although radiation therapy is often used for non-epidermodysplasia verruciformis patients with conjunctival squamous cell carcinoma, it is contraindicated in patients with epidermodysplasia verruciformis, as it has been observed to cause the development of more invasive and severe lesions.[29][35] In non-conjunctival cases of squamous cell carcinoma that have metastasized, radiation therapy may be a consideration as an adjunct to other treatments.[36](B3)
Differential Diagnosis
Important differential diagnoses to consider when evaluating epidermodysplasia verruciformis lesions include verruca-associated lesions, any lesion with hyperkeratosis and parakeratosis, seborrheic keratosis, actinic keratosis, and squamous cell carcinoma. A few cases have been reported of epidermodysplasia verruciformis that mimics pityriasis versicolor.[37] Importantly, in the case of epidermodysplasia verruciformis, the lesions present in multiples and at a young age (as early as infancy for the inherited form) or with a history of immunosuppression. In contrast, other lesions on the differential would usually present as solitary lesions or at a later age.[2]
Prognosis
The prognosis of epidermodysplasia verruciformis is complicated by the disease's lifelong nature, as lesions are often recalcitrant to conventional treatments. A substantial malignancy risk exists, estimated between 30% and 60%, predominantly associated with HPV types 5 and 8. Long-term management is necessary, focusing on rigorous photoprotection, regular dermatological surveillance for early detection of malignancies, and interventions such as surgical excision, retinoids, and immunomodulating therapies to control lesions.
Complications
Epidermodysplasia verruciformis may lead to many complications, primarily due to its chronic, progressive nature and the heightened susceptibility to specific HPV infections. The most well-known complication is the development of non-melanoma skin cancers, particularly squamous cell carcinoma, in approximately 30% to 60% of patients, often occurring in sun-exposed areas by the third or fourth decade of life. In addition to the oncogenic potential, the lesions are often recalcitrant to standard treatments, leading to persistent and widespread wart-like lesions that can significantly impair the quality of life of affected patients. The chronic and overt nature of these lesions can cause profound psychosocial distress, including social stigma, anxiety, and depression.
Consultations
Consultations for patients with epidermodysplasia verruciformis should involve a multidisciplinary approach, including dermatologists, geneticists, oncologists, infectious disease specialists, and primary care physicians. These consultations are essential for a comprehensive evaluation, accurate diagnosis, and development of a tailored treatment plan. Dermatologists can assess and manage skin lesions, geneticists can provide insights into the hereditary aspects, and oncologists can monitor and treat potential malignancies. Infectious disease specialists are critical in managing persistent HPV infections, while primary care physicians coordinate ongoing care and monitor overall health. This collaborative approach ensures holistic, patient-centered care, addressing the diverse needs of patients with epidermodysplasia verruciformis.
Deterrence and Patient Education
Patients with epidermodysplasia verruciformis should be educated about the importance of rigorous photoprotection. This includes regularly using broad-spectrum sunscreens, wearing protective clothing, and avoiding prolonged sun exposure to reduce the risk of UV light-induced skin damage and malignancies. Awareness of the signs and symptoms of skin cancer is essential, prompting immediate medical consultation if changes occur in lesions. Patients should adhere to scheduled dermatologic evaluations for the early detection and treatment of precancerous and cancerous lesions. Education on the chronic nature of the disease and available treatments can help manage expectations and improve compliance with therapeutic regimens. Additionally, providing psychological support and resources can help patients cope with the psychosocial impacts of the disease, fostering a better overall quality of life.
Enhancing Healthcare Team Outcomes
Epidermodysplasia verruciformis is a rare condition with no definitive treatment. Due to the absence of large randomized controlled trials, specific treatment strategies are not well established. This genodermatosis is best managed through a multidisciplinary team approach involving dermatologists, general surgeons, radiation oncologists, and medical geneticists. Healthcare professionals must be skilled in recognizing the characteristic skin lesions of epidermodysplasia verruciformis, conducting genetic testing for accurate diagnosis, and performing histopathological assessments to differentiate these lesions from other dermatological conditions. Comprehensive management strategies should integrate evidence-based treatments for HPV infections, dysplasia, and skin cancer prevention. Regular surveillance and early intervention are crucial to mitigate the risks associated with epidermodysplasia verruciformis.
Physicians, advanced practitioners, nurses, pharmacists, and other healthcare professionals are responsible for educating patients about epidermodysplasia verruciformis, its risks, and preventive measures. Emphasizing the importance of patient follow-up and adherence to treatment and prevention strategies is crucial, as lesions can develop, progress to malignancy, or even metastasize if left untreated.[35][36] The healthcare team should also ensure timely referrals to specialists and follow-up care to monitor disease progression. By leveraging these skills and strategies and effective interprofessional communication and care coordination, healthcare professionals can provide holistic, patient-centered care for individuals with epidermodysplasia verruciformis. This approach enhances outcomes and improves team performance in managing this complex genetic dermatologic condition.
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