Human papillomavirus infection in Netherton's syndrome

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Abstract
Background Netherton’s syndrome (NS) is a hereditary disorder with dermatological signs (e.g. ichthyosis) and a complex immunological dysfunction. In immunodeficient individuals human papillomavirus (HPV) types are associated with carcinomas on non‐mucosal sites. Objectives To study the presence of HPV infection in different skin lesions of three male NS patients and to investigate a possible association between HPV and malignancies in NS. Methods Patient 1 had extraordinary widespread multiple skin carcinomas on sunlight‐exposed areas, as well as common viral warts. Patient 2 showed disseminated viral plane warts that resolved spontaneously, and patient 3 was free of skin lesions suspicious for HPV infection; only pseudoepitheliomatous wart‐like lesions as a symptom of ichthyosis were apparent. We performed nested polymerase chain reaction analysis of DNA from benign and malignant skin lesions and HPV‐8 serology in these three patients. Results Antibodies to HPV‐8 were not detectable in our patients; however, seven of 22 (31%) biopsies of the three NS patients were positive for HPV DNA. Epidermodysplasia verruciformis (EV) ‐associated HPV types and normal cutaneous types (HPV‐2, HPV‐28) were detected. Interestingly, only the patient with cutaneous carcinomas harboured, preferentially in malignant lesions, EV‐HPV types (HPV‐19, 23, 38 and HPV‐RTRX9, closely related to EV‐HPVs), whereas plane warts of patient 2 were positive for HPV‐28. The pseudoepitheliomatous skin lesions were HPV‐DNA negative in all investigated probes. Conclusions These data in NS patients further confirm an association of EV‐HPVs with non‐melanoma skin cancer (NMSC) and suggest a possible carcinogenic role similar to that assumed for NMSC in transplant recipients. A complex immunological disorder facilitating EV‐HPV infection, negative HPV serology and photochemotherapy may all have contributed to the unusual occurrence of multiple cancers in one of our NS patients.

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