|
| ||
|
|
||
Blistering Disorders (blistering + disorders)
Selected AbstractsCholinergic control of epidermal cohesionEXPERIMENTAL DERMATOLOGY, Issue 4 2006Sergei A. Grando Abstract:, The non-neuronal cholinergic system of human epidermis includes the keratinocyte (KC) acetylcholine (ACh) axis composed of the enzymes mediating ACh synthesis and degradation, and two classes of ACh receptors, the nicotinic and muscarinic ACh receptors, mediating biological effects of the cutaneous cytotransmitter ACh. Regulation of KC cell,cell and cell,matrix adhesion is one of the important biological functions of cutaneous ACh. The downstream targets of ACh effects mediated by distinct ACh receptor subtypes include both the intercellular adhesion molecules, such as classical and desmosomal cadherins, and integrins mediating KC adhesion to a substrate. The signaling pathways include activation or inhibition of kinase cascades resulting in either up- or down-regulation of the expression of cell adhesion molecules or changes in their phosphorylation status, or both. The components of the KC ACh axis are involved in cutaneous blistering in patients with autoimmune pemphigus, junctional and dystrophic forms of epidermolysis bullosa, thermal burns, and mustard-induced vesication. Recent progress with the development of antiacantholytic therapies of patients with pemphigus using cholinomimetics indicates that cholinergic drugs may be a promising approach for other cutaneous blistering disorders. [source] A Case of Juvenile Bullous Pemphigoid, Successful Treatment with Diaminodiphenylsulfone and PrednisonePEDIATRIC DERMATOLOGY, Issue 1 2009Karen A. Marcus M.D. Because of the wide variety of bullous disorders and the considerable clinical overlap between them, it is difficult to differentiate one from the other on clinical features alone. Appropriate additional investigations are required to confirm the diagnosis. These include routine histologic examination of the skin, in addition to immunohistochemical staining and immune serology. Here, we present a rare case of juvenile bullous pemphigoid, which we will use to illustrate the difficulties encountered in the diagnostic process and to show how acquired blistering disorders of childhood should be approached. [source] A review of high-dose intravenous immunoglobulin (hdIVIg) in the treatment of the autoimmune blistering disordersCLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 2 2001S. Jolles High-dose intravenous immunoglobulin (hdIVIg) is being used increasingly for dermatological indications. Its mode of action is via a number of proposed mechanisms and it is not associated with the many side-effects of steroids and other immunosuppressive agents. The evidence for using hdIVIg in the treatment of autoimmune bullous disorders is based on uncontrolled trials and case reports. However, there are now 62 reported patients and this review aims to make a critical assessment of the current data. This has been obtained from a Medline search of the English literature from 1966 to 2000 for pemphigus vulgaris, pemphigus foliaceus, bullous pemphigoid, pemphigoid gestationis, cicatricial pemphigoid, epidermolysis bullosa acquisita and linear IgA disease. Taken together hdIVIg was effective in 81% of the patients with blistering disease. Patients appear to be more likely to respond when hdIVIg is used as adjunctive therapy (91% response rate) than as monotherapy (56% response rate). hdIVIg may offer a safe potential therapeutic avenue for resistant cases of the autoimmune bullous disorders but should be further assessed using double-blind placebo-controlled trials. [source] High-dose intravenous immunoglobulin (hdIVIg) in the treatment of autoimmune blistering disordersCLINICAL & EXPERIMENTAL IMMUNOLOGY, Issue 3 2002S. JOLLES First page of article [source] | ||||||||||