Phototherapy and photochemotherapy of sclerosing skin diseases

The treatment of sclerosing skin diseases [systemic sclerosis, localized scleroderma, lichen sclerosus et atrophicus, sclerodermoid graft-vs.-host disease, scleredema adultorum (Buschke), scleromyxedema and necrobiosis lipoidica] is difficult and remains a great challenge. Numerous treatments, some with potentially hazardous side effects, are currently used with only limited success. The introduction of phototherapy and photochemotherapy for sclerosing skin diseases has considerably enriched the therapeutic panel and proven useful in a number of sclerosing skin diseases especially in localized scleroderma. Two phototherapeutic modalitites are used for the treatment of sclerosing skin diseases, long-wave ultraviolet A and psoralen plus ultraviolet A (PUVA). This article reviews current knowledge about the application of phototherapy and photochemotherapy to various sclerosing skin disorders.     

Noninvasive procedures in dermatology. Potential infective risks and need for sterility.

Although in the area of incisional surgery the level of sterile techniques has improved in recent years and approaches that of the surgical standards in other specialties, one cannot say the same with regard to some of the nonsurgical or semisurgical procedures. Here, unfortunately, there are even no standards to aim for and no guidelines to adopt. Since in the field of nonsurgical cosmetic skin care dermatologists have a unique position, we alone must shoulder the responsibility for setting the standards and determining the appropriate conditions for carrying out these procedures. Unfortunately, it appears that in certain of the dermatologic procedures not involving actual skin incision, the level of sterility has not improved for decades. In such dermatologic procedures there are marked discrepancies between the care taken to maintain a high degree of sterility of instruments that penetrate the skin, compared with the lack of attention to sterility of instruments that do not directly penetrate the skin and the area surrounding the procedure site. It seems that the guiding principle we must strive for is to prevent the transmission of infection from one patient to another. The emphasis should be not on sterility per se but on the prevention of transmission of disease from patient to patient.     

Resistance to ischaemic nerve-fibre block in diabetes mellitus and in mitochondrial encephalomyopathies

Thermal thresholds were measured during ischaemic compression block in the left forearms of 26 healthy subjects, 10 patients with diabetes mellitus and 6 patients suffering from different kinds of mitochondrial disorders. Cold and warm thresholds in the 6 patients with deficiencies in the respiratory chain increased earlier than in normals. When cold perception was impaired, cold stimuli were perceived as warmth and pinprick perception attenuated. In diabetics cold thresholds were less elevated during ischaemic block than in controls. This was paralleled by tingling paraesthesiae in all groups. The findings show that higher resistance to ischaemic nerve-fibre block in diabetes mellitus is not exclusively based on increased anaerobic metabolism.     

X-linked chronic granulomatous disease: correction of NADPH oxidase defect by retrovirus-mediated expression of gp91-phox

Chronic granulomatous disease (CGD) is an inherited immunodeficiency resulting from the inability of an individual's phagocytes to produce superoxide anions because of defective NADPH oxidase. The disease may be treated by bone marrow transplantation and as such is a candidate for somatic gene therapy. Two thirds of patients have defects in an X- linked gene (X-CGD) encoding gp91-phox, the large subunit of the membrane cytochrome b-245 component of NADPH oxidase. Epstein-Barr virus-transformed B-cell lines from patients with CGD provide a model system for the disease. We have used retrovirus-mediated expression of gp91-phox to reconstitute functionally NADPH oxidase activity in B-cell lines from three unrelated patients with X-CGD. The protein is glycosylated and membrane associated, and the reconstituted oxidase is appropriately activated via protein kinase C. The kinetics of superoxide production by such reconstituted cells is similar to that of normal B-cell lines. These data show the potential of gene therapy for this disease.