Treatment of subepidermal immunobullous diseases.
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Child↗
Biomedical subjects
F Wojnarowska
Publications and source records attributed to F Wojnarowska.
Bullous pemphigoid and epidermolysis bullosa acquisita: presentation, prognosis, and immunopathology in 11 children.
The immunobullous diseases bullous pemphigoid (BP) and epidermolysis bullosa acquisita (EBA) are very rare in childhood. Although case studies have been detailed, there are no reports of a large series of patients documenting the effectiveness of treatment and long-term prognosis. We report the clinical presentation, immunopathologic features, disease course, and long-term prognosis of BP and EBA in a series of 11 children. The initial diagnoses based on clinical features were BP (5), EBA (3), and chronic bullous disease of childhood (CBDC) (3). These were subsequently revised from BP to EBA (2), CBDC to BP (2), and CBDC to BP or EBA (1) following the results of direct and indirect immunofluorescence and immunoblotting. Analysis of IgG subclasses in eight cases showed that the predominant subclasses were IgG1 (8) and IgG4 (6). The clinical features appeared to be highly variable, and in patients presenting with inflammatory blistering, laboratory studies were required in order to differentiate between BP and EBA. All patients improved on treatment with corticosteroids and/or sulfones, although treatment regimens showed wide variation. Their diseases tended to remit within 2 years, and their long-term prognosis was good.
Adolescent↗
Childhood vulval lichen sclerosus in a patient with ectodermal dysplasia and uncombable hair.
An 11-year-old girl was diagnosed as having ectodermal dysplasia, with abnormalities of her nails, teeth, and hair noted from infancy. Her hair was unruly and investigations confirmed the changes seen in "cheveux incoiffables"-the uncombable hair syndrome. She also had atopy requiring treatment to control asthma and eczema. When she was 8 years old she developed vulval lichen sclerosus, which was controlled easily with early treatment. The coexistence of these conditions is discussed.
Asthma↗
Upregulation of adhesion complex proteins and fibronectin by human keratinocytes treated with an aqueous extract from the leaves of Chromolaena odorata (Eupolin).
The fresh leaves and extract of the plant Chromolaena odorata are a traditional herbal treatment in developing countries for burns, soft tissue wounds and skin infections. We have previously shown that the extract had an effect on the growth and proliferation of keratinocytes and fibroblasts in culture. This study has demonstrated that Eupolin extract increased expression of several components of the adhesion complex and fibronectin by human keratinocytes. Using indirect immunofluorescence we found increased expression (dose-dependent) of laminin 5, laminin 1, collagen IV, and fibronectin. The expression of the b1 and b4 integrins was upregulated by the extract at low concentrations (0.1 and 1 microg/ml), but the expression was decreased at higher doses of Eupolin (10 microg-150 microg/ml). A number of clinical studies carried out by Vietnamese and international medical investigators have demonstrated the efficacy of this extract on the wound healing process. In this study we have shown that Eupolin stimulated the expression of many proteins of the adhesion complex and fibronectin by human keratinocytes. The adhesion complex proteins are essential to stabilise epithelium and this effect could contribute to the clinical efficacy of Eupolin in healing.
Cell Adhesion↗