The vast majority of vulvar human papilloma virus infections are produced by α human papilloma viruses and consist of exophytic or flat warts and classic or "usual" vulvar intraepithelial neoplasia. This report details 2 examples of epidermodysplasia verruciformis-like lesions of the vulva in women who were immunosuppressed. The most consistent morphologic feature was the presence of abnormal mature keratinocytes with large pale open nuclei with small nucleoli and eosinophilic cytoplasm, situated in the upper epithelial layers. In addition to these features, which are commonly seen in epidermodysplasia verruciformis-associated lesions, 1 case displayed in addition more extensively distributed abnormal nuclei, including involvement of both the upper epithelial strata and the epithelial/stromal interface. Both lesions were associated with β-papilloma virus type 5. The unique aspects of epidermodysplasia verruciformis-like lesions relative to the more common human papilloma virus infections of the vulva are highlighted and these cases illustrate the range of epithelial distribution that might be encountered in lesions involving the vulvar mucosa.
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http://dx.doi.org/10.1097/PGP.0000000000000417 | DOI Listing |
Clin Exp Dermatol
February 2024
Allergy Immunology Unit.
Background: Dedicator of cytokinesis protein 8 (DOCK8) deficiency is an autosomal recessive form of combined immunodeficiency. This rare disorder is characterized by an increased predisposition to allergy, autoimmunity and malignancies.
Objectives: To analyse clinical, immunological and molecular profiles of patients with DOCK8 deficiency.
Pediatr Dermatol
January 2024
Department of Pediatric Dermatology, HOMI Fundacion Hospital Pediatrico la Misericordia, Bogota, Colombia.
Serine/threonine kinase 4 deficiency (STK4 or MST1, OMIM:614868) is an autosomal recessive (AR) combined immunodeficiency that can present with skin lesions such as epidermodysplasia verruciformis-like lesions (EVLL). Herein, we describe a 17-year-old male patient born from consanguineous parents presenting with recurrent respiratory infections, verruciform plaques, poikiloderma, chronic benign lymphoproliferation, and Sjögren syndrome with suspected interstitial lymphocytic pneumonia.
View Article and Find Full Text PDFJ Cutan Pathol
October 2023
Department of Dermatology, Mayo Clinic, Rochester, Minnesota, USA.
J Cutan Pathol
December 2022
Department of Pathology, University of Cambridge, Cambridge, UK.
J Cutan Pathol
June 2018
Department of Dermatology, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania.
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