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WILD Syndrome

Alexander Kreuter, MD; Bettina Hochdorfer, MD; Norbert H. Brockmeyer, MD; Peter Altmeyer, MD; Herbert Pfister, PhD; Ulrike Wieland, MD; for the Competence Network HIV/AIDS

Arch Dermatol. 2008;144(3):366-372.

Background  Epidermodysplasia verruciformis (EV) is a rare genodermatosis associated with infections with specific human papillomaviruses (HPVs) belonging to the β genus of HPV. Patients with EV usually have a selective defect in cell-mediated immunity. Although skin cancer frequently develops in the sun-exposed cutaneous lesions of patients with EV, the anogenital area is usually not affected by squamous cell carcinomas related to mucosal HPV types.

Observations  We report the case of a patient with clinical similarities to EV who also presented with primary lymphedema, anogenital dysplasias, and depressed cell-mediated immunity. Swab samples and biopsy specimens from various body sites collected over a 28-month period were screened by different protocols for DNA of the HPV groups alpha, beta, and mu/nu. Seventeen -HPV types could be demonstrated. Interestingly, β-HPVs (HPV-22 and HPV-23) were detectable only in plucked eyebrows and in 1 skin swab sample. None of the specimens from lesional biopsies carried β-HPV. Consistently found -HPV types included HPV types 6, 51, 52, 61, and 84 in the genitoanal region and HPV-57 in skin lesions. Histological and cytological evaluation revealed multifocal anogenital dysplasia and benign genital and cutaneous warts.

Conclusions  To our knowledge, only 1 other similar case of an EV-like syndrome with impaired, cell-mediated immunity and primary lymphedema has been described in the literature. Based on the characteristic clinical and virological findings in the present case and the previously published case, we speculate that both patients could have a previously unknown syndrome that has clinical similarities to EV but notably differs in the associated HPV types. We suggest the acronym WILD (warts, immunodeficiency, lymphedema, dysplasia) to characterize this syndrome.

Author Affiliations: Human Papillomavirus Research Unit, Department of Dermatology and Allergology, Ruhr-University Bochum, Bochum, Germany (Drs Kreuter, Hochdorfer, Brockmeyer, and Altmeyer), and the Institute of Virology, University of Cologne, Cologne, Germany (Drs Pfister and Wieland).
Group Information: A list of the Competence Network HIV/AIDS members can be accessed at http://www.kompetenznetz-hiv.de.