Human papillomavirus infection in Netherton's syndrome.
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.


OBJECTIVE
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.
Weber
F
F
Department of Dermatology and Venereology, University of Innsbruck, Anichstrasse 35, A-6020 Innsbruck, Austria. florian.weber@uibk.ac.at
Fuchs
P G
PG
Pfister
H J
HJ
Hintner
H
H
Fritsch
P
P
Hoepfl
R
R
D000328:Adult; D004279:DNA, Viral / Q000032:analysis; D006801:Humans; D007057:Ichthyosis / Q000150:complications*; D007154:Immune System Diseases / Q000150:complications; D008297:Male; D009894:Opportunistic Infections / Q000150:complications; D027383:Papillomaviridae / Q000145:classification / Q000302:isolation & purification*; D030361:Papillomavirus Infections / Q000150:complications*; D011230:Precancerous Conditions / Q000821:virology; D012878:Skin Neoplasms / Q000821:virology*; D013577:Syndrome; D014412:Tumor Virus Infections / Q000150:complications*
D004279:DNA, Viral