|Questions and Discussions
Epidermodysplasia verruciformis (EV) is a rare genodermatosis
associated with a high risk of skin cancer (Ramoz et al., 2000).
EV results from an abnormal susceptibility to specific related
human papillomavirus (HPV) genotypes and to the oncogenic potential
of some of them, mainly HPV5. Infection with EV-associated HPV
leads to the early development of disseminated flat wart-like
and pityriasis versicolor-like lesions. Patients are unable
to reject their lesions, and cutaneous Bowen carcinomas in situ
and invasive squamous cell carcinomas develop in about half
of them, mainly on sun-exposed areas.
The lesions often resemble verrucae planae (Sullivan and Ellis,
1939). Malignant degeneration, usually of the superficial basal
cell type, is frequent. Characteristic changes in the epidermal
cells with peculiar vacuolization are observed. Ellis (1953)
stated that this disorder occurs most frequently in Orientals.
Avoidance of sun exposure would certainly help to reduce her
risk of malignant transformation. There has been reports on
the use of systemic retinoids and interferons A in treating
these cases but they tend to recur when the dose is reduced
Please share your views on the management of this patient.
Wonder if light curettage or CO2 laser would be appropriate
as a modality to remove the seb keratoses on her face?
|Comments from Faculty and Members
Tan, Heng Soon MD, Brigham and Women's Primary Care
Associates at Newton Corner, Harvard Medical School, Boston,
MA, USA on May 27, 2005
She should be approached like a patient with selective immunodeficiency
susceptible to HPV infection. The key would be to administer
an antiviral agent that will suppress HPV infection. There are
clinical trials on an HPV vaccine to prevent genital HPV and
cervical cancer. Perhaps a Medline search on HPV vaccine will
point to the principal investigators who may be able to advise
Benjamin Barankin MD, Department of Dermatology, University
of Alberta, Edmonton, Canada on May 28, 2005
I would try one treatment at a time in as hidden an area as
possible. First, I would consider imiquimod to one of the lesions;
if it works, the cosmetic result would likely be the best. Otherwise,
I would consider a trial of isotretinoin to see how she does,
and if there is benefit, see if it can be maintained with topical
retinoids. Otherwise, cryotherapy for 5 seconds followed by
curettage to the lesions may help. Again, due to her skin color,
I would choose a test site first.
Janjua Shahbaz MD, Lalamusa, Pakistan on
May 29, 2005
Strict sun avoidance to minimise the risk of possible cutaneous
malignancies is very important for this patient. I have observed
very good response to topical tretinoin to treat verruca plana.
Topical DNCB 10% has also been used with promising results in
extensive cases. I do not recommend Cryo to treat any lesions
on face (due to risk of scarring).
Choon Siew Eng FRCP, Department of Dermatology, Hospital
Sultanah Aminah, Johor Bahru, Malaysia on May 30, 2005
This case is very interesting with SK-like lesions. Saw HPE
of two cases when I was in UK. Both cases occurred in HIV patients
with pityriasis versicolor-like lesions. The HPE is very distinctive
and after you see one case, you will not forget.
Your patient is only 19 year-old, with HPV5 and staying in our
country. Personally I would spend a fair bit of time telling
her the risk of non-melanoma skin cancer and getting her to
use sunscreen and protective clothing diligently.
The SK-like facial lesions can be easily and rapidly removed
by either CO2 laser, radiosugery or even currettage if those
are the only lesions she is keen to get rid off. Guess all her
lesions would benefit from oral retinoids, preferrably 13 cis-retinoic
acid but it is a headache giving retinoids to our teenage patients
because in our country contraception is frowned upon. However,
if you are considering putting her on oral retinoids, it would
be interesting to see whether these SK-like lesion also melt
away. Saw only two patients in my practice in Malaysia. One
in Kota Bahru and one here referred by surgeon after removal
of SCC. Lost to follow-up.
Khalifa Shaquie MD, PhD, Professor of Dermatology,
College of Medicine, University of Baghdad, Baghdad, Iraq
on May 30, 2005
We have a focus of EV in Sammara City, Iraq because of marriages
between close relatives. The clinical pictures are similar to
this presented case. The seborrheic warts-like usually change
to either aggressive SCC or BCC and usually the lesions are
around the eyes. Many cases ended with eye enucleation (this
going to be published). Similar to EV ,we have observed cases
among patients with immunocompromised immunity especially patients
with kidney transplant so called acquired EV with high incidence
of skin malignancy and viral warts on the sun exposed areas.
The management of EV is very difficult and usually all measures
fail to control the lesions.
Amy S. Paller, M.D. Walter J. Hamlin Professor of Dermatology
and Pediatrics, Feinberg School of Medicine Northwestern University,
Chicago, IL, USA on May 30, 2005
Although physical destruction can be used, it is painful and
laborious -- can lead to scarring. We would probably see if
she could mount a reaction to squaric acid dibutyl ester immunotherapy
- probably coupled with high dose cimetidine as we start to
slowly treat the warts by physical means - cryo would be preferred.
Wonder if 5-FU might be worth a try to a field area as well?
Steve Tyring MD, PhD, Clinical Professor of Dermatology,
University of Texas Medical School, Houston, TX, USA on
May 30, 2005
My suggestion for this patient would be oral acitretin (or
isotretinoin); there is little basis for choosing one retinoid
over the other in this case. At the same time, I suggest that
she use topical imiquimod nightly. Although either therapy might
have limited activity by itself, I have observed that retinoids
and imiquimod act additively (and sometimes synergistically).
The reason for the additive/synergistic benefit is that retinoids
upregulate interferon-response elements in the cell, e.g. STAT.
Imiquimod induces interferons alpha and gamma. Therefore, retinoids
allow the body to better respond to the interferon induced by
If some of the lesions do not response as well as others, you
may wish to remove the less-responsive lesions (e.g. laser).
The combination of the retinoid and imiquimod will reduce the
chances of a recurrence.