Extremely low levels of epidermal skin-derived antileucoproteinase/elafin in a patient with impetigo herpetiformis
Autor: | H.F.C. Rulo, J.J.A. Peperkamp, P.C.M. van de Kerkhof, J. Schalkwijk, A.L.A. Kuijpers, E.M.G.J. de Jong |
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Rok vydání: | 1997 |
Předmět: |
Pathology
medicine.medical_specialty integumentary system biology Epidermis (botany) business.industry De rol van proteasen en proteaseremmers bij epidermale differentiatie en ontstekingsprocessen Elastase The role of proteinases and proteinase inhibitors in epidermal differentiation and inflammation Dermatology medicine.disease Proteinase 3 Enzyme inhibitor Psoriasis Immunology Generalized pustular psoriasis biology.protein Medicine business Impetigo herpetiformis GeneralLiterature_REFERENCE(e.g. dictionaries encyclopedias glossaries) Elafin |
Zdroj: | British Journal of Dermatology, 137, 123-129 British Journal of Dermatology, 137, pp. 123-29 British Journal of Dermatology, 137, 1, pp. 123-129 British Journal of Dermatology, 137, 123-29 |
ISSN: | 0007-0963 |
Popis: | Summary Impetigo herpetiformis (IH) is a rare pustular dermatosis with unknown aetiology, typically occurring during pregnancy. Based upon a similar clinical and histological presentation, i.e. spongiform accumulation of polymorphonuclear leucocytes in the stratum corneum, several authors consider IH as a variant of generalized pustular psoriasis (GPP), while others state that IH is a separate entity. Skin-derived antileucoproteinase (SKALP) is a strong and specific inhibitor of human leucocyte elastase (HLE) and proteinase 3, two neutral proteinases that have been implicated in leucocyte migration and tissue destruction. Previously, we reported decreased SKALP activity in pustular forms of psoriasis compared with plaque psoriasis. In this study we present a case study of a patient with IH, where SKALP activity was measured using biochemical and immunochemical techniques. Epidermal scales and sera were collected during the course of the disease. Comparison was made with three patients with GPP and six patients with plaque psoriasis. Initially, anti-HLE activity in epidermal scales of the patient with IH was comparable with values in patients with GPP, i.e. decreased compared with plaque psoriasis. During the course of the disease, anti-elastase activity dropped to undetectable levels, concomitant with the appearance of free elastase activity. This finding suggests a total saturation of epidermal anti-HLE activity. Low levels of SKALP, presumably complexed with HLE, could be measured immunochemically in scale extracts. Serum levels of total SKALP correlated with the disease activity. We suggest that a reduced amount of epidermal SKALP contributes to an imbalance between elastase and its inhibitor, resulting in the formation of epidermal pustules. This mechanism of pustule formation could apply both to GPP and IH, suggesting a final common pathway in the pathogenic mechanisms of IH and GPP. |
Databáze: | OpenAIRE |
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