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 département 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.
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 epidemiological of rare skin cancer diagnosed in the département 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 3rd 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.
In 1913, a distinctive clinical entity of acute genital ulcer occurring in an adolescent girl with a non-venereal infectious aetiology was described by Lipschütz. Since the initial description, ...several aetiologies have been discussed, and among them, paratyphoid fever is very uncommon. After her return from a trip, a 25-year-old girl developed high fever and diarrhoea. Examination of the vulva revealed a genital ulcer. The rest of the general examination was normal. Blood cultures identified Salmonella paratyphi A, and a diagnosis of Lipschütz's ulcer associated with paratyphoid fever was made. Bacteriaemia was then treated with antibiotics and the vulvar ulceration rapidly disappeared. Lipschütz described a distinctive clinical entity of acute genital ulcers occurring in adolescents. To our knowledge, we report herein the second case associated with proved paratyphoid fever. The authors thus recommend that typhoid or paratyphoid fever should be included in the differential diagnosis of genital ulcerations.
PDF
