Objective: Inherited keratinizing disorders are a spectrum of relatively rare skin diseases with a variety of congenital defects in the keratinizing process. In this study, we evaluated patients with inherited keratinizing disorders in the Tokyo urban area from the clinicians' viewpoint.
Patients: We retrospectively evaluated 77 cases of inherited keratinizing disorders (male/female: 43/34 cases, mean age at first visit: 20.2 years) who visited our institution in the past 5 years.
Methods: The cases were classified into 3 major groups; ichthyoses, palmoplantar keratodermas (PPK), and macular- and punctuate-type keratodermas. Definite diagnoses were achieved according to the clinico-pathological features and genetic analyses.
Results: The ichthyoses group (48 cases: 62.3%) consisted of dominant ichthyosis vulgaris (IV: 13 cases), X-linked ichthyosis (XLI: 16 cases), lamellar ichthyosis (LI: 4 cases), and bullous/nonbullous congenital ichthyosiform erythroderma (BCIE and NBCIE: 3 cases each). PPK groups (21 cases: 27.3%) included Vörner-type (7 cases) and Nagashima-type (9 cases). Macular/punctuate-type keratodermas (8 cases: 10.4%) included 4 cases of Darier's disease. The discrepancy between percentages above and reported incidence of each disorder most likely resulted from the increasing tendency of visits in patients with severe symptoms. The therapeutic approach was application of topical moisturizers, in combination with topical vitamin D3 analogue, steroid, and antibiotics/antifungal agents. In addition, some cases were treated with oral retinoids and antihistamines.
Conclusions: Although genetic analysis is necessary for definitive diagnosis, this simplified classification based on clinical features and morphological changes seems to be useful for clinical diagnosis and first-line aid for patients. The establishment of guidelines for diagnosis, therapy and patient care of inherited keratinizing disorders in Japan is urgently needed.
Objectives: The present study evaluated the significance of LEED under different ablation speeds based on histopathology and mid-term results.
Materials and Methods: A total of 97 endovenous laser ablations (EVLAs) were performed by 1,320 nm pulsed YAG laser to incompetent great saphenous veins (GSVs). LEED was fixed by 120 J/cm, and ablation speeds were selected as 0.5 mm/sec in the low-speed group (n=42) and 1 mm/sec in the high-speed group (n=55). System parameters were set by power output of 6 W, peak power of 343 W, pulse width of 350 nsec and frequency of 50 Hz. In the high-speed group, ELVA was repeated twice with the same parameter after high ligation.
Results: Mean diameter of GSV was 9.4mm and high ligation was performed in 87 legs (90%). Mean LEED was 120 ± 3 J/cm in the low-speed group and 119 ± 11 J/cm in the high-speed group. No laser-related complications occurred for any procedures. All treated GSVs were occluded by thrombus formation one month after EVLA. Negative remodeling rate shown by proportion of veins that shrunk more than 50% to total ablated veins was observed in 6% of the high-speed group and 48% of the low-speed group after one month. These values were 11% and 62% after 3 months, while both groups showed equivalent shrinkage of 70% and 75% after 6 months and 98% and 100% after 12 months, respectively.
Conclusions: LEED is a standard parameter to obtain successful EVL;however, ablation speed affects the GSV shrinkage in mid-term observation. A low speed of 0.5 mm/sec with LEED of 120 J/cm accelerated the healing of ablated GSV without any complications.
Serum autoantibodies against p53 (p53Ab) have been identified in patients with various tumors and are used as one of molecular markers. Here we examined the possible application of p53Ab for diagnosis of breast cancer. We collected sera from 141 patients with breast cancer and determined the p53Ab level by enzyme-linked immunosorbent assay with MESACUP anti -p53 test ELISA kit. The positive rate of p53Ab was greater than those of current molecular markers, breast cancer antigen -225 (BCA225), carcinoembryonic antigen (CEA), and carbohydrate antigen 15-3 (CA15-3). We found no significant relationships between the p53Ab level and patient's clinical characteristics including age, tumor size, and metastasis. Further, the value of p53Ab did not correlate with other serological tumor markers and expression levels of hormonal receptors (estrogen receptor and progesterone receptor) and HER2. The sensitivities of BCA225 and CEA were relatively low in early stages and increased in a stage-dependent manner while the positive rate of p53Ab was sustained from early to late stages at a high level (14.6% and 15.4%, respectively). Combinations of p53Ab with BCA225, CEA, and CA15-3 improved the positive rate to 20.6%, 21.2%, and 26.7%, respectively, which were greater than the positive rate obtained by combinations of other serological markers. Thus, serum p53Ab is a useful molecular marker of breast carcinoma and we recommend the combination assay with other serological markers for clinical diagnosis.