Rare skin cancer: a population-based cancer registry descriptive study of 151 consecutive cases diagnosed between 1980 and 2004.

BACKGROUND: There are few epidemiological data available on rare skin cancer, including Merkel cell carcinoma, Paget's disease, adnexal carcinoma, and sarcoma. We conducted this study to investigate the epidemiology of rare skin cancer diagnosed in the department of Doubs from 1980 to 2004. METHODS: Data were collected from a population-based cancer registry from 1980 to 2004. Diagnosis was based on the 3(rd) edition of the International Classification of Diseases for Oncology. The incidence rates were standardized on world population. RESULTS: One hundred and fifty one patients were investigated (88 women and 63 men). Median age for the diagnosed disease was 63 years. The standardized incidence rate was 0.82/100 000 person-year (95% CI = 0.68-0.96) and increased from 0.25 in 1980-1984 to 1.50 in 2000-2004. Fifty nine cases (39%) were sarcomas, 35 (23%) adnexal carcinomas, 27 (18%) Merkel cell carcinoma and 27 (18%) Paget's disease. The standardized incidence rates were 0.37/100 000 (0.27-0.47) for sarcomas, 0.16 (0.10-0.22) for adnexal tumors, 0.13 (0.08-0.18) for Merkel cell carcinoma, and 0.15 (0.09-0.21) for Paget's disease. CONCLUSIONS: Our results based on a population-based cancer registry showed an increase of the standardized incidence rate for all types of rare skin tumors. These results may be useful when considering the growing interest in rare diseases in identifying risk factors and planning scientific research programmes.

Preventive effect of ultraviolet radiation on murine chronic sclerodermatous graft-versus-host disease.

Although previous studies have demonstrated the efficient modulatory effects of ultraviolet radiation B (UVB) on cutaneous graft-versus-host disease (GVHD), most animal research on GVHD has been performed in murine models of acute GVHD. Here, we studied the preventive effect of UVB radiation on the occurrence of chronic sclerodermatous (Scl) GVHD in a murine model. Scl GVHD was induced by transplanting lethally irradiated BALB/c mice with B10.D2 bone marrow and spleen cells. Recipient mice were exposed to UVB before or after bone marrow and spleen cell infusion. Histological and clinical evaluation of GVHD was performed, in association with the characterization of epidermal Langerhans cells. UVB irradiation of recipients after, and more remarkably before, transplantation induced a decrease of Scl GVHD severity associated with epidermal Langerhans cells depletion. We conclude that UVB irradiation of recipient before or after transplantation has a preventive effect on cutaneous Scl GVHD and may represent an effective strategy for prevention of Scl GVHD.

Cervical intraepithelial neoplasia associated with epidermodysplasia verruciformis HPV in an HIV-infected patient: a manifestation of immune restoration syndrome.

We report a case of a cervical intraepithelial neoplasia associated with epidermodysplasia verruciformis human papillomavirus (HPV) type 5 and HPV type 16 in a human immunodeficiency virus-infected patient. Furthermore, epidermodysplasia verruciformis-like cutaneous eruptions after initiation of highly active antiretroviral therapy has never been described as a manifestation of an immune restoration syndrome.

Primary cutaneous lymphomas: a population-based descriptive study of 71 consecutive cases diagnosed between 1980 and 2003.

Although primary cutaneous lymphomas (PCL) are the second most common group of extra-nodal non-Hodgkin lymphomas, few epidemiological data are available in the literature, and most of them are provided by large databases from population-based cancer registries in the US or patients attending a single institution. We conducted this study to investigate the epidemiological and clinical features of PCL diagnosed in the department of Doubs from 1980 to 2003. Data were collected from the Doubs cancer registry from 1980 to 2003. Seventy-one patients with PCL were investigated. 82% were cutaneous T-cell lymphoma (CTCL) and 18% were cutaneous B-cell lymphoma (CBCL). Among CTCL, mycosis fungoides (MF) represented 58% and Sezary syndrome 10%. The standardised incidence rate of PCL was 0.42 for 100 000 person-years and significantly increased from 0.21 in 1980-1984 to 0.70 in 2000-2003 (p <0.05). The incidence rate of CTCL was 0.34 for 100 000 person-year and significantly increased from 0.2 to 0.57 (p <0.05). For MF and CBCL, the incidence rates were 0.20 and 0.08, respectively and did not vary significantly from 1980-1984 to 2000-2003. Five-year survival was 64.5% for PCL patients similar to MF patients. Our results provide updated data on the incidence of PCL in France.

Use of amniotic membrane transplantation in the treatment of venous leg ulcers.

Amniotic membrane (AM), the most internal placental membrane, has unique properties including antiadhesive effects, bacteriostatic, wound protection and pain-reduction properties, as well as epithelialization initialization capacities. Furthermore, AM is widely available and less costly than other bioengineered skin substitutes. In a prospective pilot study, we evaluated the safety, feasibility, and the effects on healing of AM graft in 15 patients with chronic venous leg ulcers. AM grafts were prepared from placentas harvested during cesarean section. All grafted AM had adhered to the wound bed 7 days after being applied with a 100% engraftment rate. The percentage of granulation tissue increased significantly (from 17% on day 0 to 69% on day 14, p<0.0001), along with a significant decrease of fibrinous slough (from 36% at day 0 to 16% at day 14, p<0.001). A significant clinical response occurred in 12 patients (80%) including complete healing (20%) in three during the 3-month follow-up period. The ulcer surface area decreased significantly from a mean value (+/- standard deviation) of 4.59 +/- 2.49 cm(2) at baseline to 2.91+/-2.01 cm(2) on day 30 (p<0.001). All patients experienced a significant reduction of ulcer-related pain rapidly after AM transplantation. No adverse events were recorded. AM transplantation seems to function as a safe substrate, promoting proper epithelialization while suppressing excessive fibrosis. Further advantages of biotherapy with AM are its easy and low-cost production, and that it can be applied as an ambulatory treatment without immobilization. AM transplantation may thus be considered to be an alternative method for treating chronic leg ulcers.