Recent advances in understanding the etiology and treatment of systemic sclerosis (SSc) were overviewed. In vivo overexpression of connective tissue growth factor (CTGF) mRNA has been demonstrated in dermal fibroblasts in patients with SSc. Serum CTGF levels are elevated in SSc patients and are correlated with the extent of skin and lung fibrosis. Thus, CTGF appears to be the key molecule in the pathogenesis of the disease. Fetal microchimerism is another issue in the pathogenesis of SSc. Recent studies have demonstrated that fetal microchimerism was more frequent in women with SSc than in normal women. Additional investigations are needed to verify the role of microchimerism in the pathogenesis of the disease. The effects of low-dose and high-dose D-penicillamine (D-Pen) were compared in a double-blind, randomized, controlled study. High-dose D-Pen was not more effective than low-dose D-Pen, suggesting that D-Pen itself may not be effective at all. Photopheresis (extracorporeal photochemotherapy) may have limited effects, but the results of a recent crossover trial were disappointing. Low-dose relaxin was effective in a double-blind, placebocontrolled study, but high-dose relaxin was not more effective than placebo. Epoprostenol (prostaglandin I2) seems to be an effective agent for treating isolated pulmonary hypertension in patients with SSc.
We studied 12 patients to examine whether or not iontophoresis of prostaglandin E1 is effective in treating Raynaud’s phenomenon associated with systemic lupus erythematosus, systemic sclerosis, or mixed connective tissue desease. Prostaglandin E 1 was applied iontophorentically at the following two different frequencies : in 6 patients who had relatively mild symptoms, iontophoresis was applied once a week for 6 weeks (group A). In another 6 patients who had more severe symptoms iontophoresis was applied twice a day either for 1 or 2 weeks (goups B and C : three patients for each), followed by once a week for the subsequent 6 weeks. The following factors were assessed at the end of treatment and 6 weeks after the discharge : coldness, numbness pain, frequency of Raynaud’s phenomenon, hemokinesis test, and doctor’s clinical evalution. Complete remission was obtained in 5 of 6 patients in group A, 2 of 3 in group B, and 1 of 3 patients in group C. Therapeutic effects were still observed 6 weeks after the discharge in all patients. No adverse side effects were observed throughout the observation period in any patient. Therefore, iontophoresis with prostaglandin E 1 is potentially useful in treating Raynaud’s phenomenon with collagen-vascular deseases.
We sent a questionnaire to 793 students ranging from the sixth grade of elementary school to nursing or medical school. Our study revealed that 58.6% of the students suffered from acne vulgaris. The average onset was 13.3 years of age. Acne lesions appeared on the forehead first and later developed on the cheek and jaw. The patients often had seborrhea and a family history of acne. More than half of them stated that lack of sleep was one of the exacerbating factors. More than half of them stated that larger number of exacerbating factors. Elementary and junior high school students and males reported that sweating caused deterioration in their acne. Adverse effects of cosmetics on acne were denied by 56% of the students. More than half of the university and college students noticed a relationship between menstrual cycle and acne lesions, especially premenstrual exacerbation. Most of them tried to control skin lesions by OCT topical agents, better sleep, or squeezing acne lesions with their fingers. Only 11.8% of them visited doctors, and 67.5% of those treated by doctors were satisfied with the treatment.
We surveyed the member institutions of the Japan Psoriasis Association in order to elucidate (1) the subjects of concern for the deeper understanding of the pathogenesis of psoriasis and (2) the current treatment modalities for psoriasis in Japan. The results showed that gene analysis and discovering the immune desorders surrounding cytokines are the two main areas of interest in clarifying its pathogenesis. Current popular treatment modalities are (1) oral cyclosporin and (2) oral etretinate for systemic therapies and (1) topical vitamin D3 application, (2) topical steroid application, and (3) topical PUVA treatment for local treatments. Combination therapies consist of (1) low dose oral etretinate with topical steroid or (2) low dose oral etretinate with topical PUVA treatment. The results of this survey reflect the current general knowledge of the pathogenesis and the treatment of psoriasis.
A 56-year-old female gave birth to a child with CCAVB 27 years ago. Fifteen years ago, the patient was diagnosed as having Sjogren’s syndrome upon her first visit to our clinic based on her sicca symptoms, ophthalmic findings, sialography, positive antinuclear antibody, and positive anti-SS-A/SS-B antibodies ; we have obserbed the patient since that time. Three years ago, the staining pattern of the antinuclear antibody changed from a speckled pattern to nucleolar one, and her anti-SS-A/SS-B antibody titers began to decrease gradually. Raynaud’s phenomenon and sclerodactyly developed two years ago, and the scleroderma progressed to both arms, face, trunk, and legs despite treatment with vasodilators. Other organ involvements such as pulmonary fibrosis and reflux esophagitis confirmed the diagnosis of systemic sclerosis. Her scleroderma has improved remarkbly with oral PSL (20 mg/day) therapy.
Two cases of Von Recklinghausen’s multiple dermal neurofibromas were treated with direct injection of absolute ethanol, into small neurofibromas after local anesthesia. The volume of one injection of absolute ethanol was around 0.1~0.3 ml. After repeated injections, which were done once every two or three weeks, the sizes of the neurofibromas clearly decreased. Histopathological examination of biopsied neurofibroma tissues that had been injected with ethanol revealed massive necrosis of the tumor nests. Side effects were intense pain during ethanol injection. The benefits of this treatment are that it can be done in an outpatient clinic basis and that initial development of growth of small neurofibromas can be effectively inhibited.