fig 1 and 2 showing seborrheic keratoses-like lesions on the face

fig 3

figs 4, 5 and 6 showing pityriasis versicolor-like lesions and planar warts on upper back and palm respectively

Laboratory Data

Facial Seborrheic Keratoses like lesions in Epidermodysplasia verruciformis

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 or stopped.

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?

1. Roncalli de Oliveira W, Neto CF, Rady PL, Tyring SK.Seborrheic Keratosis-like lesions in patients with epidermodysplasia verruciformis. J Dermatol. 2003 Jan;30(1):48-53.
2. Kwon OS, Hwang EJ, Bae JH, Park HE, Lee JC, Youn JI, Chung JH. Seborrheic keratosis in the Korean males: causative role of sunlight. Photodermatol Photoimmunol Photomed. 2003 Apr;19(2):73-80.
3. Li YH, Chen G, Dong XP, Chen HD. Detection of epidermodysplasia verruciformis-associated human papillomavirus DNA in nongenital seborrhoeic keratosis Br J Dermatol. 2004 Nov;151(5):1060-5.
4. Tomasini C, Aloi F, Pippione M. Seborrheic keratosis-like lesions in epidermodysplasia verruciformis. J Cutan Pathol. 1993 Jun;20(3):237-41.
5. Ramoz N, Taieb A, Rueda LA, Montoya LS, Bouadjar B, Favre M, Orth G. Evidence for a nonallelic heterogeneity of epidermodysplasia verruciformis with two susceptibility loci mapped to chromosome regions 2p21-p24 and 17q25.J Invest Dermatol. 2000 Jun;114(6):1148-53.
6. Sullivan, M.; Ellis, F. A. : Epidermodysplasia verruciformis (Lewandowsky and Lutz). Arch. Derm. Syph. 40: 422-432, 1939.
7. Anadolu R et al, Treatment of epidermodysplasia verruciformis with a combination of acitretin and interferon alfa-2a. J Am Acad Dermatol 2001;45:296-9.

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 you.

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 the imiquimod.

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.