[Urticarias and angioedemas: the mediators].
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Biomedical subjects
Publications and source records attributed to J Sayag.
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Tryptase (T), chymase (C), carboxypeptidase A, cathepsin G-like constituent of preformed mediators contained in mastocyte granules, are a group of neutral proteases with proteolytic activity. These enzymes gives differentiation of two groups of mastocytes, MCTC and MCT as a function of the richness of enzymes. Although the functions of these molecules are becoming better and better understood, their exact roles as well as that of their inhibitors, still remain to be explored in urticaria.
The endothelial cells, because of their privileged situation between the tissues and the vessels, play an important role in the inflammatory reaction. Expression of adhesion molecules (ELAM 1 especially) allows adhesion of circulating neutrophils to the epithelial cell and thus initiates the reaction. During the reactions of immediate hypersensitivity, the flow of leucocytes observed in the late phase is dependent on mastocytes, the recruitment being under the positive or negative influence of several cytokines.
Urticaria and physical angioedema frequency is hard to evaluate in children. In this series, we keep 53/1000. Physical urticaria is found in 36% of the cases and concerns cholinergic urticaria, urticaria to cold, idiopathic dermographism and mastocytosis, and delayed urticaria to pressure. The authors insist on the interest of the etiologic research in these forms of infantile urticaria.
Mastocytosis in children are very polymorphic, most often remaining isolated, without an associated visceral lesion. Beside the phenomenon of "urtication" characteristic, papulo-nodular lesions of urticaria and angio-oedema during the mastocytosis are rarely met. In contrast, congestive signs with pruritus, dermographism, congestive erythema and general signs are frequent.
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A 45 year-old woman with no history of familial disease presented with multiple cutaneous and cerebral cavernous angiomas. The diagnosis rested on biopsy for cutaneous angiomas and on MRI for cerebral angiomas. The clinical manifestations of cerebral angiomas were headaches and a cerebellar syndrome, the latter most probably due to progressive growth of cerebellar angiomas. Systematized cavernous angiomatosis is characterized by multiple angiomas of the brain and other viscera and is considered to be a phacomatosis.
Papular skin eruptions are uncommon in lupus erythematosus (LE), and their occurrence may suggest several diagnoses. We report four cases of papular eruptions in LE patients. Two of these patients had purely lupoid papules on acute LE in one and on chronic LE in the other. In the remaining two cases the papules were formed by mucinous deposits that were either secondary to LE or belonged to an associated primary mucinosis. These four cases prompted us to discuss the significance of papular eruptions in lupus erythematosus. LE-specific papular manifestations have a purely lupoid histological and immunopathological substratum. In systemic LE, the frequency of these manifestations varies from one series to another, but they seem to rank fourth after vespertilio, alopecia and photosensitivity. The papules vary in size and number, and they usually complicate a severe and active systemic LE. In chronic LE, a papulo-nodular eruption may be observed, but this is even rarer. Papules occurring in the course of LE may suggest a diagnosis of mucinosis, this disease being either secondary to, or associated with LE. Secondary mucinosis is intricated with LE-specific histological abnormalities. It appears as a symptomatic alcianophilic deposit induced by cytological alterations in some dysimmune collagen diseases, such as dermatomyositis or LE. In the literature, three cases of mucinosis secondary to LE are well documented, and to these we add a fourth case in which the papules contained lupoid lesions and a variable amount of dermal mucinous deposit. The literature has also yielded sixteen cases of primary papular mucinosis associated with systemic LE (12 cases) or chronic LE (4 cases); we add to these a case of systemic LE in which the papular eruptions varied in course, density and size of the papules. As in isolated papular mucinosis, histology regularly shows a copious mucinous deposit. When present, the LE-specific skin manifestations are clearly distinct from those of mucinosis (except for the above-mentioned cases of papular lupus). The course of papular mucinosis usually runs parallel to that of LE which is characterized by its severity and its articular, haematological and renal manifestations. Analysis of the literature also provides various anatomico-clinical elements which help in differentiating between isolated papular mucinosis and primary papular mucinosis associated with LE. In the majority of cases treatment relies on corticosteroid therapy or synthetic antimalarial agents, but the results are irregular since LE seems to be more resistant to treatment than mucinosis.(ABSTRACT TRUNCATED AT 400 WORDS)
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The changes induced by ageing of the skin are particularly visible in photo-exposed areas, indicating the interaction between actinic factors and factors specific to the ageing process itself. The corresponding histological signs affect various structures: epidermis (thinning of the epidermis, reduced cell proliferation...), dermis-epidermis junction (disappearance of the microvilli with defective adhesion of the epidermis to the dermis), dermis (degeneration of elastin and collagen, which is photoinduced, and a moth-eaten appearance with loss of microfibrils as a result of time-related ageing), microcirculation (reduced vascularization, particularly visible in photo-exposed areas...) appendages.
Oxatomide (Tinset) is a new, powerful, antiallergic agent. Pharmacological and morphological studies have shown that oxatomide is not only an antihistaminic agent but it also inhibits mast cells and basophils degranulation, and neoformed mediators synthesis and release. Open and comparative studies have shown oxatomide to be an effective agent in the treatment of chronic urticaria including physical urticaria and food allergy. All these studies confirm its constant efficacy, giving good to excellent results in 68 to 89 percent of cases. It compares favorably with the classic anti-H1, particularly in that the symptomatic response is much quicker. Moreover the drug is always well tolerated, even when used at higher doses than those presently recommended (30 mg twice daily).
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