Epidermodysplasia verruciformis. A case associated with primary lymphatic dysplasia, depressed cell-mediated immunity, and Bowen's disease containing human papillomavirus 16 DNA

A Human Papillomavirus–Associated Disease With Disseminated Warts, Depressed Cell-Mediated Immunity, Primary Lymphedema, and Anogenital Dysplasia

“…(5) Our patient showed no signs of premalignant changes in the sunexposed areas, which frequently appear in persons with EV between the ages of 30 and 40 years. 5 To our knowledge, the combination of the clinical symptoms seen in the present patient has only been reported once before, by Ostrow et al 9 In their patient, a 37-yearold white man without family history of lymphedema and warts, congenital lymphatic disease was also present on all 4 extremities, and disseminated flat warts and pityriasis versicolor-like papules developed during adolescence. Further features shared with our patient were anergy to routine skin testing, depressed mitogen-stimulated lymphocyte transformation, severe CD4 T-cell and B-cell depletion, low albumin and low total serum protein levels, and condylomatous (partially dysplastic) lesions.…”

“…Although the clinical findings have similarities to EV, the presented case differs in several decisive points: (1) The HPV types of the EV-defining species ␤-HPV, including HPV types 5,8,9,12,14,15,17,[19][20][21][22][23][24][25]36, 38, and 47, were absent in all analyzed biopsy specimens. This, and the lack of histologic features characteristic of EV, excludes the diagnosis of EV in our patient.…”

“…Twenty years ago in the Archives, Ostrow et al 9 reported the case of a patient with clinical features of EV and 2 additional findings that are not characteristic of patients with EV: congenital lymphatic dysplasia and HPV-16-positive carcinoma in situ of the thumb. Moreover, their patient had a history of squamous cell carcinoma of the foot and the groin presenting as large, ulcerative lesions, again not typical conditions for patients with EV.…”

“…Further features shared with our patient were anergy to routine skin testing, depressed mitogen-stimulated lymphocyte transformation, severe CD4 T-cell and B-cell depletion, low albumin and low total serum protein levels, and condylomatous (partially dysplastic) lesions. The patient described by Ostrow et al 9 had an in situ carcinoma of the thumb and a history of squamous cell carcinoma of the groin. Virological analyses in their patient showed that EV-defining ␤-HPVs were also absent.…”

BritishAssociation ofDermatologists' guidelines for the management of squamous cell carcinomain situ(Bowen's disease) 2014

“…117,[125][126][127][128][129][130][131][132][133][134][135][136][137][138] HPV-associated digital SCC suggests the possibility of genital digital spread as a mechanism of tumour induction, 115 and patients and partners should be followed up for digital and genital HPV-associated lesions. 138 Management of periungual SCC is usually local excision, Mohs micrographic surgery or distal phalanx amputation.…”