Abstract | November 9, 2021
Epidermodysplasia Verruciformis – Tree Man Disease: A Rare Skin Disorder
Learning Objectives
- Describe and identify Epidermodysplasia verruciformis which is an extremely rare disorder associated with formation of tree bark like verrucous skin lesions. Learners will also get to identify unique case where the acquired variant of EV is seen in an immunocompetent person as traditionally EV has been associated with immunosuppressed condition.
- Discuss about the pathogenesis of EV which includes creation of conditions facilitating replication of HPV. In the inherited form, there is mutation in TMC 6 and TMC 8 genes which helps in HPV multiplication. In the acquired form, immunodeficiency facilitates viral multiplication. Because of this, acquired EV is usually reported in patients with acquired cell-mediated immunodeficiency like HIV-positive patients or in patients on immunosuppressive treatment such as organ transplant recipients.
- Also, EV commonly affects people under 20 years of age, but rarely it may occur later. Our patient started developing progressive “tree bark like lesions” when she was in her 40s.
Introduction: Epidermodysplasia verruciformis (EV), also known as “tree man disease” is an extremely rare skin disorder with unknown prevalence and a total of around 501 patients described worldwide. EV is characterized by abnormal susceptibility of the skin coating to human papillomaviruses (HPVs). There are 2 forms of EV- classic inherited and late onset acquired forms, both of which create conditions to facilitate replication of HPV. In the acquired form, immunodeficiency facilitates viral multiplication.
Case Presentation: We report a case of 48-year-old immunocompetent African American female who presented with generalized weakness with bilateral lower extremity chronic pain and multiple verrucous lesions leading to functional disability. Her symptoms started with bilateral leg swelling in 2015 that progressively worsened over a year leading to the development of typical “tree bark like lesions”, that rendered her bedbound since then. She does not have any immunodeficiency disorders and denied family history of similar skin lesions or history of consanguineous marriages. Physical exam showed multiple, diffuse, dry, non-tender, black wart-like lesions extending from knees to feet. Bilateral lower extremity sensation was diminished to both pin prick and light touch. Peeling off a warty lesion caused bleeding. WBC on presentation was 8600 with 63% neutrophils and 26% lymphocytes. Hemoglobin, serum electrolytes and blood glucose were within normal limits.
Diagnosis: Diagnosis of EV was established by skin biopsy which showed histological findings of hyperkeratosis, acanthosis and vacuolated cells in epidermis and presence of HPV virus.
Management: There are no curative therapies for EV, management is conservative. Our patient began physiotherapy as inpatient and was advised to maintain hygiene and adequate hydration and not walk barefoot. Upon discharge to rehab-facility she was advised to adopt sun protection measures to prevent development of non-melanoma skin cancer. During follow up after two weeks, she was found to be functionally doing better.
