Keratinocytes biosynthesize glucosylceramides, acylglucosylceramides and sphingomyelins, which are potential precursors of ceramides in stratum corneum. Specificly, acylglucosylceramides are unique components comprised of both very long chain fatty acids as the amide-linked fatty acids and as esterified fatty acid linked through the ω-hydroxy group. The one found in the human epidermis consists of two acylglucosylceramide groups, one containing sphingenine and the other containing phytosphingosine with a single double bond. The linoleic acid content as ester-linked fatty acids is very high in human epidermis. We named these acylglucosylceramides contain linoleic acid “epidermosides”; they are key molecules of the epidermal permeability barrier. Epidermosides are the precursors of acylceramides and ω-hydroxy ceramides ; the latter are the major components of the corneocyte lipid envelope. Glucosylceramides and sphingomyelins have structural heterogeneities in the ceramide portion. All ceramide species are derived from glucosylceramides, while a fraction of Cer 2 and Cer 5 come from sphingomyelins.
Patients with Behcet’s disease (BD) (35 patients ; 18 males and 17 females) who visited our clinic from 1990 to 1999 were clinically analyzed. The diagnosis was based on the Japanese Clinical Criteria. The patients with the incompletetype of BD were significantly higher in number than those with the complete type (55% vs. 29%, respectively). Oral aphthosis, which has been frequently reported as an initial symptom of BD, was present in most of our patients during their clinical courses (94%). Furthermore, there was a marked difference in the frequency of the oral aphthosis between males and females (72% vs. 41%, respectively). The positive rate for HLA-B51 allele was exceedingly high among the patients with clinically complete-type disease (66%), suggesting a pathogenic background for BD. NSAIDs, antibiotics including tetracycline and macrolide, steroid, and cyclosporin were frequently administered during the last half decade ; in particular, systemic steroid therapy was chosen for patients with ocular involvement as well as general symptoms (i.e. multiarthralgia and high grade fever). The results indicate that the steroid therapy may be beneficial not only for rapid suppression of the clinical activity of BD, but also for an avoidance of possible adverse effects caused by multi-drug intervention.
Super LizerTM (SL) is a linear polarized light therapeutic instrument which provides a high output infrared ray. In this study, sixty patients over 18 years of age with various types of alopecia areata (AA) were topically irradiated by SL, and its effect as a therapeutic method was evaluated. AA consisting of single or multiple patchy lesions was irradiated for three minutes intermittently once per one or two weeks. Alopecia totalis and universalis cases were irradiated for five minutes continuously on the occipital side of the scalp once daily for four weeks in combination with topical/systemic corticosteroid and general PUVA therapies. Nineteen out of 22 cases (86.4%) of AA with a single patchy lesion were cured, while 10 out of 18 cases of AA with multiple patchy lesions (55.6%) showed hair regrowth in irradiated areas 1.4 months earlier than those of the non-irradiated areas (by X2, p=0.0002). In the cases of alopecia totalis and universalis, no obvious difference in hair regrowth was observed between the irradiated and non-irradiated areas after the treatment. As adverse effects caused by SL treatment, two patients complained of heat sensations in the irradiated area. These findings suggest that SL, with its non-invasive properties, is a useful apparatus for the treatment of mild forms of AA.
We reported a case of squamous cell carcinoma arising in an epidermal cyst. The patient was an 87-year-old woman who had a fifty year history of a mass on her left buttock. The lesion had gradually enlarged during the previous year. An excisional biopsy was done. Histpathologically, there was a cystic lesion composed of benign squamous epithelium with a transition to squamous cell carcinoma. Malignant degeneration of an epidermal cyst is uncommon.
Pilomatricoma is the most common hair-follicle tumor. In 1965, Cantwell and Reed first reported an association of pilomatricoma with myotonic dystrophy. Since then, many cases have been reported. We report a case of unusual, multiple, giant pilomatricomas in a Japanese 35-year-old man. His younger sister also had congenital myotonic dystrophy. On first examination, he had five lesions in all on the scalp, trunk, and right upper arm. Three of them were 1.5×1 cm, 2×1.5 cm, and 3.1×1.5 cm subcutaneous nodules on the scalp and right upper arm. Two of them were 90×56 cm, and 31×15 mm pediculated giant tumors on the chest and right upper arm. These lesions had enlarged over about 3 or 4 years. Physical examination revealed the typical clinical features of myotonic dystrophy such as male pattern baldness, cataract, atrophy of the sternocleidomastoid, grip myotonia, and percussion myotonia. All of the tumors were renewed, and all of them were found to be benign pilomatricomas.