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Objective: To determine the incidence, prevalence, survival rates, clinical manifestations, and longterm outcome of patients with morphea (localized scleroderma) and its subtypes over a 33 year period in Olmsted County, Minnesota., Methods: We used the unique data resources of the Rochester Epidemiology Project to review all Olmsted County medical records with any potential diagnosis consistent with morphea (including plaque, generalized, bullous, linear, and deep entities) from 1960 through 1993., Results: We screened 1030 medical records and identified 82 (59 female; 23 male) cases of morphea first diagnosed between 1960 and 1993. All cases were followed until death or migration from Olmsted County, a total of 754 person-years of observation. The annual age and sex adjusted incidence rate per 100,000 population was 2.7 (95% confidence interval 2.1, 3.3). The incidence rate increased significantly over the 33 years (p = 0.0037) on an average of 3.6% per year. The prevalence (estimated using cumulative incidence) at 80 years of age was about 2/1000. 50% of the patients had a cutaneous softening or evidence of disease resolution by 3.8 years' duration. The shortest active disease duration was found in the plaque group (50% resolution or skin softening by 2.7 years) compared to 5.5 years in the deep group. Arthralgias, synovitis, uveitis, and joint contractures were more frequent in the linear and deep categories. Although 9 patients (11%) developed some disease related disability over the followup period, this was common (44%) in the deep group. No case of morphea developed severe internal organ involvement and none progressed to systemic sclerosis. The survival rate was not significantly different from the general population (p = 0.409)., Conclusion: Morphea, and its subtypes, are more common than previously recognized, and can lead to important disability.

Objective: To address the subsequent risk of internal cancer in a population-based cohort of patients with Bowen's disease., Design: Incident cases of skin cancers other than malignant melanoma occurring in a defined population were classified as basal cell carcinoma, squamous cell carcinoma, or Bowen's disease. Through medical records, all patients were followed up for the development of subsequent internal cancer until they died, moved from Rochester, Minn, or January 1, 1986, whichever came first. Incidence rates of skin cancer and subsequent cancer were computed; the subsequent rate of internal cancer was compared with that prevailing in the community., Patients: Enrolled were all permanent residents in the population base of Rochester, Minn, who developed basal cell carcinoma (n = 657), squamous cell carcinoma (n = 169), or Bowen's disease (n = 71) on the basis of pathologic examination and clinical presentation from 1976 through 1984., Main Outcome Measure: The relative risk of subsequent internal cancer among patients with Bowen's disease compared with that of the population base from which they arose was 1.1 (95% confidence limits, 0.5, 1.6)., Results: Annual incidence rates, adjusted to the 1980 US white population, were 14.9 per 100,000 for Bowen's disease, 38.8 for squamous cell carcinoma, and 146 for basal cell carcinoma. The estimated relative risk for subsequent cancer was 0.9 (95% confidence limits, 0.5, 1.6) among patients with squamous cell carcinoma and 1.0 (95% confidence limits, 0.7, 1.3) for patients with basal cell carcinoma. These risks were not significantly different for various durations of follow-up or for sun-exposed vs sun-protected sites., Conclusions: We find no evidence in these population-based cohort data of and increased risk of subsequent internal cancer associated with Bowen's disease or other forms of nonmelanomatous skin cancer.

Between 1970 and 1984 in Rochester, Minnesota, rare skin cancers developed in 15 local residents: cutaneous T cell lymphoma (six subjects), dermatofibrosarcoma protuberans (four), adenocarcinoma of sweat glands (two), Merkel cell carcinoma (one), liposarcoma (one), and extramammary Paget's disease (one). These cases were identified through a unique computerized retrieval system that is maintained at the Mayo Clinic for the population of Rochester, Minnesota. The annual incidences of these cancers in the Rochester population were 0.9, 0.5, 0.3, 0.2, 0.2, and 0.2 per 100,000 residents, respectively (standardized to 1980 U.S. population). To our knowledge, this is the first report of the incidences of these rare skin cancers in a well-defined population.

Population-based annual incidence rates for histologically proved basal cell carcinoma were derived from the records linkage system of the Rochester Epidemiology Program Project. Residents of Rochester, Minnesota, during the years 1976 to 1984, formed the observational cohort. A total of 657 first episodes of basal cell carcinoma were observed. Annual incidence rates per 100,000 persons in the Rochester population, standardized to the 1980 U.S. white population, were 175 for men, 124 for women, and 146 combined. Recurrent or subsequent basal cell carcinoma was observed among 30% of patients during an average of 4.5 years of follow-up; no metastatic lesions occurred. The rates of recurrence were similar after complete excision or treatment with curettage and cauterization in these observational data.

The records linkage resources of the Rochester Epidemiology Program Project were used to identify 169 incident cases of invasive squamous cell carcinoma of the skin in the population of Rochester, Minn, between 1976 and 1984. The overall age-standardized incidence was 38.8/100,000 persons (63.1 in men; 22.5 in women). Metastasis occurred in 3.6%, and during an average of 3.8 years of follow-up, recurrence was seen in 3.6% while subsequent new lesions occurred in 12%. The demographics and course of patients with squamous cell carcinoma were different from either those of Bowen's disease or basal cell carcinoma in these population-based data.

A population-based epidemiologic study of herpes progenitalis was conducted in residents of Rochester, Minnesota, for the 15-year period 1965 through 1979. Three hundred ninety-two patients (129 males, 263 females) were identified. The average annual incidence rate of herpes progenitalis was 50 per 100,000 population. A continuous increase in incidence was demonstrated throughout the study period (P less than 0.0005, trend test). In 1979, the incidence peaked at 128 per 100,000 population. The median age at onset was 24 years, and more than 80% of the patients were 18 to 36 years of age. Females predominated by 1.5:1 (P less than 0.001) and were, on the average, 4 years younger than the male patients. Although 15 pregnancies in the series were affected by herpes progenitalis, no neonatal herpes was noted, nor was the number of unfavorable outcomes of pregnancy unusual. No invasive or in situ genital carcinoma developed in patients after 1,544 person-years of follow-up.