Nihon Hifuka Gakkai zasshi. The Japanese journal of dermatology最新文献

The HLA class II antigens in 33 Japanese pemphigus patients were investigated by both serologic and restriction fragment length polymorphism (RFLP) analyses: 17 cases of pemphigus vulgaris (PV), 13 cases of pemphigus foliaceus (PF), 3 cases of unclassified pemphigus. In serologic typing, DR2 was absent in PV. DR5, DRw6, DRw12, and DRw52 were positively associated with PV. DQw1 was positively associated with PF. RFLP analyses showed that DRw6 PV patients had a disease-associated restriction fragment representing DQw5, the same association as that found in DRw6 Jewish PV patients. On the other hand, all 13 PF patients were serologically typed for DQw1, which could not be further subdivided into DQw5 by RFLP analyses. These results suggest that Japanese and Jewish PV patients may be immunogenetically closely related to each other, but Japanese PV patients appear to be immunogenetically different from Japanese PF patients.

A 22-year-old woman, whose first infant had died of lethal junctional epidermolysis bullosa (JEB), requested prenatal diagnosis for her third pregnancy. At 20 weeks gestation, fetal biopsy was performed under direct vision by fetoscopy. A semithin section of epon-embedded skin showed dermo-epidermal separation at the light microscopic level. Electron microscopy revealed the site of separation to be within the lamina lucida of the epidermal basement membrane (EBM). Indirect immunofluorescence on a 5 microns cryostat specimen of skin showed a complete absence of GB3 monoclonal antibody immunostaining at the EBM compared with a control 18 week old normal fetal skin sample. The diagnosis was therefore made that the fetus was affected with lethal JEB and a prostaglandin termination performed. The diagnosis was confirmed by further studies on the aborted fetus. 54 cases of prenatal diagnosis of various types of epidermolysis bullosa performed at Institute of Dermatology over the last 10 years are briefly reviewed. Several social and practical problems to launch prenatal diagnosis in Japan are also discussed.

In order to reveal the cytological nature of polyploid cells, the cell suspension of squamous cell carcinoma was separated into low density (1.050 greater than), intermediate density (1.050 to 1.088), and high density (1.988 greater than) fractions, by density gradient centrifugation. The DNA content of the tumor cells in each fraction were measured on the smear specimens prepared by Giemsa's staining and Feulgen's stainings. As the results, it was found that the cells showing high NC ratio and having high DNA content were observed in the high density fraction. However, there was no specific relationship between the nuclear contour index and density of the tumor cell.

Radiographic skeletal examinations were performed in sixteen adult patients who had received etretinate therapy for various keratinizing disorders (psoriasis vulgaris 13, pustular psoriasis 2, pustulosis palmoplantaris 1) over periods ranging from 0.7 to 4.5 years. The total dose of etretinate ranged from 5.1 to 36.5 g. In these patients, the frequency and the degree of skeletal alterations including hyperostosis, calcification of ligaments and periosteal thickening was found to be higher than that in age- and -sex matched, nontreated controls. Furthermore, almost all of these patients were asymptomatic and revealed no abnormalities in laboratory data, including levels of serum calcium, inorganic phosphate and alkaline phosphatase. It is necessary for patients who are undergoing long-term etretinate therapy to be examined regularly for such skeletal abnormalities.

Recently, it has been shown that 1,25 (OH)2 Vitamin D3 (VD3) shows growth inhibition and enhances differentiation of keratinocytes in vitro. To elucidate the further effect of VD3 on keratinocytes, we studied the effect of recombinant human gamma interferon (IFN-gamma) and VD3 on the expression of HLA-DR antigen and MHC class I antigen on 3 normal and 6 transformed keratinocytes by means of FACS analysis. IFN-gamma (500 JRU/ml, 72 hr) induced the expression of HLA-DR antigen on 3/3 normal keratinocytes, 3/4 trichilemmoma and 1/2 squamous cell carcinoma cell lines. When these cells were cultured with both IFN-gamma and VD3 at the same time, the expression of HLA-DR antigen was significantly decreased. Sequential treatment of these cells with IFN-gamma and VD3 (each 72 hr), or vice versa, showed significant suppression of HLA-DR antigen expression. The fluorescens intensity of class I antigen was increased after IFN-gamma treatment, however, this effect is inhibited when the cells were treated with combination of IFN-gamma and VD3.

Eosinophilia-myalgia syndrome (EMS), a new connective tissue disease was reported and named in 1989 from New Mexico, U.S.A. L-tryptophan has been suspected as the causative agent of EMS. This L-tryptophan was made in Japan, but no definite case of EMS has been reported in Japan. We report 2 patients with EMS. A 72-year-old woman and a 74-year-old woman, who had been treated with oral L-tryptophan 1 g/day for 4 and 5 months by the same doctor simultaneously. The clinical courses of the two cases were similar. A diffuse erythema and swelling appeared on the arms and spread over the whole body. The skin lesions turned into lustrous sclerosis. Eosinophilia was remarkable in the early stage. ANA, DNA anti-body and ENA antibodies were all negative. Neurological examination revealed a mild peripheral neuropathy. No sclerodactylia and no Raynaud's phenomenon in our cases are characteristic findings in EMS. The simultaneous onset of two cases using the same drug at the same time implicates the close relation of L-tryptophan ingestion to EMS.

Recently secretory IgA (S-IgA) was found to be secreted from the eccrine gland. By using a sandwich enzyme immunoassay, we measured the concentration of S-IgA 1) in sweat (sweat S-IgA) and 2) in the extract buffer obtained by pipetting++ on the skin (skin surface S-IgA). Skin surface hydration and skin surface lipid were measured at the sites where the samples of skin surface S-IgA were collected. (These measurements were taken immediately after the buffer was pipetted). The quantity of both sweat S-IgA and skin surface S-IgA differed according to the sites where they were collected. Sites in order of decreasing sweat S-IgA level: face, chest, forearm. Sites in order of decreasing skin surface S-IgA level: face, chest, palm, forearm, sole. (Skin surface S-IgA levels on palm and forearm were approximately equal). Although the amount of skin surface S-IgA was not related to the skin surface hydration, there was a significant correlation between skin surface S-IgA and skin surface lipid.

A 15-month-old girl had an asymptomatic eruption of yellowish-brown flat papules of 12 months' duration. Those papules were seen multiply on her face and in a small number on her neck and trunk. Skin biopsy showed a massive infiltrate of histiocytic cells in the upper and middle dermis. Electron microscopically, these cells had comma-shaped bodies in the cytoplasm, and formed desmosome-like junctions at the cell peripheries. A diagnosis of benign cephalic histiocytosis was made on the basis of these clinical, histological, and ultrastructural findings. To the best of our knowledge, this is the first report of benign cephalic histiocytosis in Japan.

A case of 23 years old man with idiopathic pachydermoperiostosis is reported. He showed cutis verticis gyrata, clubbed fingers and periosteal new bone formation without any causative basic disorder. His serum level of FSH, LH, estradiol and estriol were elevated, but their significance was not clear. Histological examination of the skin from the forehead revealed sebaceous hyperplasia and dermal thickening, where deposit of alcian blue and colloidal iron positive substance were detected. The deformed forehead and eyelids were corrected by plastic surgery. One hundred and twenty one cases of pachydermoperiostosis reported so far in Japan are briefly reviewed. Most of them were male (94.1%), about one fourth had a family history. The principal features are: clubbing of the digits (88.4%), periosteal new bone formation (94.1%), coarsening of the facial features with furrowing of the skin of the face (72.7%) and cutis verticalis gyrata (59.5%). Arthralgia (40.5%), hyperhidrosis of the feet and hands (44.6%), gastric hypertrophy (8 cases), gastric ulcers (5 cases) and endocrine abnormalities (17 cases) were also reported.