Epidermodysplasia Verruciformis After Hematopoietic Stem Cell Transplantation in a Patient With Severe Combined Immunodeficiency Syndrome

Am J Dermatopathol. 2021 May 1;43(5):e65-e67. doi: 10.1097/DAD.0000000000001918.


Epidermodysplasia verruciformis (EV) is a rare dermatologic disorder that is characterized by skin-colored-to-light brown flat, discrete or confluent papules resembling verruca plana. EV is divided into 2 forms: a classical genetic form and an acquired form. Classical genetic EV is caused by mutations in EVER1 and EVER2 genes. Acquired EV develops in immunocompromised patients such as HIV-positive patients and transplant recipients. Patients with a prior history of hematopoietic stem cell transplantation (HSCT) have tendency to develop generalized verrucosis. We report an extraordinary case of disseminated epidermodysplasia verruciformis seen in a 7-year-old boy diagnosed with severe combined immunodeficiency syndrome who had undergone HSCT. He had plane, brown papules involving his face, forearms, neck, anterior chest, nape, back, and knees. Cutaneous biopsy showed typical characteristic findings of EV: large cells with gray-blue cytoplasm and keratohyaline granules of different sizes in the granular and spinous layers. Herein, we present an unusual case of disseminated EV in a HSCT patient with typical histopathologic findings and treatment options.

Publication types

  • Case Reports

MeSH terms

  • Child
  • Epidermodysplasia Verruciformis / immunology*
  • Epidermodysplasia Verruciformis / pathology*
  • Hematopoietic Stem Cell Transplantation / adverse effects*
  • Humans
  • Immunocompromised Host*
  • Male
  • Severe Combined Immunodeficiency / therapy*