In this study, we adopted the AA investigational assessment guidelines proposed by Olsen et al. The patients with AA were divided into four groups by age of onset of AA [childhood (age: 0–15), adolescence (age: 16–19), early-adulthood (age: 20–39), and late-adulthood (age: 40≦)], and then analyzed. The study population (total 205 cases) included 136 females (66.3%) and 69 males (33.7%) with a male/female ratio of 1: 1.9. The age of onset ranged from 1 to 73 years. The mean age of onset was 29.7±17.1 years in males and 34.5±17.6 years in females (p=0.0704). One-hundred twenty (58.5%) out of 205 patients had limited alopecia (S1–2, less than 50% scalp hair loss); 61 patients (29.8%) had moderate alopecia (S3–4, 50–99% scalp hair loss); 5 patients (2.4%) had alopecia totalis; and 19 patients (9.3%) had alopecia universalis. The distribution of cases by sex and age of onset showed two peaks between 20 and 24 years and between 50 and 54 years in females, while no significant peaks were seen in males. The month of onset was identified in 147 out of 205 patients, and the distribution of cases by month of onset showed that the incidence of onset increasd in April and decreasd in November. One-hundred and five (71.4%) out of 147 patients were included in the 7 months from April to October. The childhood group consisted of 18 females and 19 males with a female/male ratio of 0.95:1; the adolescence group consisted of 9 females and 5 males with female/male ratio of 1.8:1; the early-adulthood group consisted of 57 females and 24 males with female/male ratio of 2.4:1; and the late-adulthood group consisted of 52 females and 21 males with female/male ratio of 2.5:1, suggesting that the rate of female/male ratio may be high in the late onset cases (p=0.0876). The rate of extensive alopecia (S3–4≦) was 56.8% in the childhood group, 64.3% in the adolescence group, 43.2% in the early-adulthood group, and 27.4% in the late-adulthood group, suggesting that patients with early onset showed more severe types (p=0.0057). Positive family histories of AA were seen in 46 (22.4%) out of 205 patients, and the positive rate of a family history of AA was significantly higher in females than that in males (male 13.0%, female 27.2%, p=0.0216). The positive rate of a family history of AA was 27.0% in the childhood group, 21.4% in the adolescence group, 24.7% in the early-adulthood group, and 17.8% in the late-adulthood group (p=0.6631). The rate of positive past and/or present histories of atopic dermatitis (AD) was 40.5% in the childhood group, 35.7% in the adolescence group, 23.5% in the early-adulthood group, but there were no patients with positive histories of AD in the late-adulthood group, suggesting a high incidence of AD in the patients with early onset (p<0.0001). The rate of positive anti-thymoglobulin antibody was 13.5% in the childhood group, 21.4% in the adolescence group, 17.3% in the early-adulthood group (p=0.5019), and 24.7% in the late-adulthood group. In this study, it was suggested that the clinical features of AA are distinguished by sex and the age of onset, and it may reflect heterogeneity of the mechanisms in the development of AA.
We report a case of a 10-year-old boy with Laugier-Hunziker-Baran syndrome. He had pigmented, oval or round macules with well defined borders on the lip as well as the tip of his finger. These pigmented macules were first noticed 8 years previously. On examination, no intestinal polyposis or adrenal dysfunction suggestive of Peutz Jeghers syndrome or Addison’s disease was noted. A biopsy of a pigmented macule from the lip revealed melanin pigmentation of the basal layer with occasional melanophages in the upper dermis. In Japan, 53 cases of Laugier-Hunziker-Baran syndrome have been reported. We reviewed the complications and the treatment and discussed the difference between Laugier-Hunziker-Baran syndrome and Peutz-Jeghers syndrome from the point of view of the literature and our case.
Mohs micrographic surgery is the most popular and effective technique for the removal of high-risk skin cancers in Europe and the United States. Conversely, wide local excision is frequently used in Japan instead of Mohs micrographic surgery. The purpose of this study was to evaluate the surgical margin of basal cell carcinomas, squamous cell carcinomas and Bowen’s diseases after Mohs micrographic surgery compared with conventional surgery. In over 90% of total tumors, the required mean surgical margin was 4 mm. This was smaller when compared to previously described recommended surgical margins in Japan. Understanding the appropriate predictive surgical margin in relation to each tumor is important for surgical planning and the creation of guidelines for excision margins in Japan.
To examine the current treatments for Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) in Japan, we retrospectively analyzed reports of 43 cases of SJS and 54 cases of TEN published in medical journals from 2000 to 2005. Corticosteroid was administered systemically in most cases, and methylprednisolone (mPSL) pulse therapy (1,000 mg/day) or mini pulse (less than 600 mg/day) was selected for 14 cases (32.6%) of SJS and 28 cases (51.9%) of TEN. Combinations of plasmapheresis or high-dose immunoglobulin therapy with corticosteroid therapy were performed in 5 cases of TEN and 3 cases of TEN. In 2 cases of TEN in which the symptoms continued to progres in spite of using corticosteroids and high-dose immunoglobulin therapy, plasmapheresis was performed additionally and was effective in their recovery. This suggests that the combination of these 3 treatments may be useful in seriously ill patients. The mortality rate of patients with SJS was 2.3%, and with TEN was 7.4%. We also reviewed the deceased cases published in medical journals from 2000 to 2005. One case of SJS and 4 cases of TEN were reported.