title: Cutaneous Neoplasms in Myotonic Dystrophy Type 1.
abstract: BACKGROUND
  The most frequent skin features associated with myotonic dystrophy type 1 (DM1) are frontal alopecia and pilomatrixomas. Several reports suggest that the incidence of basal cell carcinoma is increased in DM1. However, two recently published studies examining this topic have contradictory results.
  
  
  OBJECTIVE
  To retrospectively study the incidence of cutaneous tumours in patients with DM1.
  
  
  METHODS
  The clinical features of 102 Caucasian patients diagnosed with DM1 at Bellvitge Hospital in Barcelona, Spain, were retrospectively analysed. Clinical charts of the patients were reviewed, and cutaneous tumours diagnosed in our hospital were recorded. A group of 103 Caucasian patients matched for age and sex were used as the control group.
  
  
  RESULTS
  A total of 56 male and 46 female patients with DM1 were included in the study (mean age 49.07 years, SD 13.02). At least 1 basal cell carcinoma was diagnosed in 6 patients in the DM1 group versus 3 patients in the control group (p = 0.332). The mean age at diagnosis of the first basal cell carcinoma was 51 years compared with 66 years in the control group (p = 0.035). Five patients with DM1 presented pilomatrixomas versus none in the control group (p = 0.029). We did not detect any melanoma in our DM1 patients.
  
  
  CONCLUSIONS
  Basal cell carcinomas appeared at a significantly younger age in our DM1 patients than in the general population, and this suggests that, at least in some patients, DM1 may predispose to the development of basal cell carcinomas.
authors.affiliation: Department of Dermatology, Hospital Universitari de Bellvitge, IDIBELL, University of Barcelona, Barcelona, Spain.
authors.forename: Joaquim
authors.initials: J
authors.lastname: Marcoval
authors.forename: Montserrat
authors.initials: M
authors.lastname: Olivé
authors.forename: Montserrat
authors.initials: M
authors.lastname: Bonfill-Ortí
authors.forename: Laura
authors.initials: L
authors.lastname: Martínez-Molina
authors.forename: Ana
authors.initials: A
authors.lastname: Talavera-Belmonte
mesh_terms: D000328:Adult; D000368:Aged; D004198:Disease Susceptibility; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009223:Myotonic Dystrophy / Q000150:complications*; D012189:Retrospective Studies; D012878:Skin Neoplasms / Q000175:diagnosis* / Q000209:etiology; D055815:Young Adult
keywords: Basal cell carcinoma; Cutaneous neoplasm; Myotonic dystrophy; Skin; Steinert disease