[Impact of antioxidative therapy on state of enzymic redox-system of glutathione in patients with phlegmons of maxillofacial area].

Traditional and antioxidative therapy (retinol and alpha-tocopherol) was done by therapeutic course to 18 patients with phlegmons of maxillofacial region. In the peripheral blood leucocytes and tissues (skin, muscle) of the patients with phlegmons there were determined glutathione reductase activity, glutathione peroxidase activity and content of glutathione. Disbalance of glutathione enzyme redox system in leucocytes of patients with phlegmons was corrected by comprehensive therapy after 1 week. Spirman's correlation analysis showed positive correlation between glutathione depended enzymes in skin and muscle of patient with phlegmons before and after therapeutic course.

Photoprovocation test and immunohistochemical analysis of inducible nitric oxide synthase expression in patients with Sjögren's syndrome associated with photosensitivity.

BACKGROUND: Annular erythema (AE) in Sjögren's syndrome (SS) usually develops on areas of sun-exposed skin and is exacerbated during summer. OBJECTIVES: To evaluate photosensitivity in SS and to investigate the involvement of ultraviolet (UV) radiation in the development of AE in SS. METHODS: Phototesting with UVA and UVB was performed on 14 SS patients, including 10 with primary SS. Clinical and histological features as well as expression of inducible nitric oxide synthase (iNOS) in the evoked skin lesions were compared with those of lupus erythematosus (LE). Eleven SS patients had a history of photosensitive AE (n = 4), papules (n = 3) or other types (n = 4) of lesions on their sun-exposed skin that were induced or aggravated by sunlight exposure. RESULTS: Phototesting induced a prolonged erythematous response (n = 8), infiltrated erythema (IE) (n = 4) and/or papules (n = 3) in 11 of 14 SS patients, including one with primary SS without a history of photosensitivity. Histologically, the induced IE and papules showed coat-sleeve-like or sparse perivascular infiltration of lymphocytes similar to that in primary skin lesions of AE in SS. No epidermal changes characteristic for LE were found except for partial and mild liquefaction degeneration in three cases. In contrast, two cases were indistinguishable from the papular type of polymorphic light eruption in several aspects, including their primary skin lesions and early response to a photoprovocation test. Immunohistochemistry revealed diffuse expression of iNOS throughout the epidermis, which is characteristic for LE, in the three SS patients with minimal liquefaction degeneration, while the remaining seven SS patients examined exhibited no iNOS staining or a normal expression pattern. CONCLUSIONS: Our results indicate that photosensitivity exists in certain primary SS patients, and that UV is critical to the development of AE in SS, probably through a pathological mechanism distinct from that in LE.

Decreased activity of acid alpha-glucosidase in a patient with persistent periocular swelling after infusions of hydroxyethyl starch.

After infusion therapy with hydroxyethyl starch (HES) on account of sudden hearing loss, a 68-year-old woman developed a marked and persistent periocular swelling. This extraordinary adverse effect caused us to search for an explanation by means of histopathology, immunohistochemistry, immunoelectron microscopy and biochemistry. In lesional periocular skin and in normal-appearing skin, lysosomal storage of HES could be detected with a specific HES antibody in histiocytes, endothelial cells, basal keratinocytes and small nerves. In the periocular skin, a stronger deposition of HES was found in addition to distinct xanthomatous changes as well as features of lymphoedema. In view of lysosomal HES storage we measured the pH-dependent activity of the lysosomal alpha-glucosidase (GAA) in cultured fibroblasts. We found a 50% decreased activity of the acid GAA, which is consistent with a heterozygous state of glycogenosis type II (Pompe's disease) and potentially of pathogenetic relevance for the intralysosomal accumulation of HES. Xanthomatous changes and lymphoedema are likely to be secondary effects, but contribute considerably to the clinical manifestation of persistent visible swelling. This observation could point to a role for GAA in the elimination of tissue-stored HES. Patients with decreased activities of GAA may be at risk of unusual adverse effects following extraordinary and prolonged tissue storage of HES, especially if it is infused in large quantities.