|
| ||
|
|
||
Bullous Dermatosis (bullou + dermatosi)
Selected AbstractsEpidermolysis bullosa acquisita and neuroendocrine pancreatic cancer , Coincidence or patho-genetic relationship?JOURNAL DER DEUTSCHEN DERMATOLOGISCHEN GESELLSCHAFT, Issue 10 2007Jan-Ole Busch Summary The etiology of epidermolysis bullosa acquisita (EBA) is unknown. EBA may be associated with other autoim-mune systemic diseases; it also has been described in connection with different malignant tumors, showing complete remission after successful treatment of the tumor. In such cases, EBA may be regarded as a paraneo-plastic dermatosis. We detected a highly differentiated neuroendocrine pancreatic cancer in a 78-year-old woman with EBA. Even thought her tumor was completely removed and the patient has been disease-free for over seven years, a complete regression of her autoimmune bullous dermatosis could not be induced. By using intravenous immunoglobulins in combination with mycophenolate mofetil, further blister formation could be ameliorated. [source] Unique epidermolytic bullous dermatosis with associated lethal cardiomyopathy related to novel desmoplakin mutationsJOURNAL OF CUTANEOUS PATHOLOGY, Issue 5 2009Angeliki Asimaki Background:, Desmoplakin plays a vital role in cell adhesion, linking the transmembrane desmosomal complex to the cytoskeletal network. Clues to the biological significance of desmoplakin have emerged from the embryonic lethal phenotype of null mice and from naturally occurring human desmoplakin mutations, which cause cardiocutaneous phenotypes. Index case:, In this study, we describe a child who presented with the unique constellation of bullous dermatosis, profound plantar keratoderma, alopecia totalis and cardiomyopathy leading to sudden cardiac death at the age of 9 years. Results:, This complex cardiocutaneous phenotype is associated with compound heterozygosity for two novel nonsense desmoplakin mutations. Histological examination of a plantar skin biopsy showed full thickness epidermal acantholysis with superimposed spongiosis, hyperorthokeratosis and focal parakeratosis. Immunohistochemistry and quantitative confocal microscopy showed abnormal tissue distribution and reduced levels of expression for plakoglobin, desmoplakin and connexin 43 at epidermal junctional sites. Conclusions:, Interpretation of the changes in the context of the two mutations provides insight into the mechanism of clinical cell adhesion disease. [source] Clinical and immunopathological heterogeneity of 22 cases of linear IgA bullous dermatosisJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 9 2008M Sobjanek [source] PL1 Subepithelial bullous diseases , topic overviewORAL DISEASES, Issue 2006M Mravak-Stipeti Subepithelial bullous diseases comprise the group of mucocutaneous autoimmune blistering diseases characterized by subepithelial separation and the deposition of immunoglobulin and complement against several antigens along the basement membrane zone (BMZ). This result in spectrum of diseases that affect skin, oral mucosa, and other mucosal membranes and include bullous pemphigoid (BP), mucous membrane (cicatricial) pemphigoid (MMP), linear IgA disease (LAD), and chronic bullous dermatosis of childhood (CBDC). The most common clinical features are oral erosions, desquamative gingivitis and conjunctival fibrosis, as well as skin lesions, predominantly in older female population. The heterogeneity of clinical presentation and diversity of target autoantigens have contributed to difficulties in characterizing this condition immunologically. In addition to the clinical presentation and a subepithelial vesicle or bullae on routine histologic analysis, the diagnosis is based on direct and indirect immunofluorescence studies. The nature of the disease is determined by the target antigens in the epithelium and BMZ such as antigen 180 (BP180), antigen 230 (BP230), laminin 5, and beta 4 integrin. Circulating IgG and IgA antibodies bind to different epitopes of BP180. The use of salt-split skin substrate enables differentiation between epidermal and dermal 'binders'. Since the antigen and the antibody titer appear to have direct relationships with the disease severity, and a combination of clinical finding and antibody titer provides valuable prognostic data, these investigations should be carried out routinely. Clinicians should recognize clinical spectrum of SBD, the histopathologic and immunopathologic characteristics, the differential diagnosis, the treatment, and the natural history of the disease. Involvement of oral medicine specialists, dermatologists, ophthalmologists, otolaryngologists and gastroenterologists contribute to early diagnosis and will aid in providing SBD patients with the highest quality of care. [source] Linear IgA bullous dermatosis induced by interferon-, 2aCLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 5 2009P. Kocyigit Summary Linear Ig A bullous dermatosis (LABD) is an acquired autoimmune subepidermal blistering disorder with linear deposits of IgA along the basement membrane zone. Its cause is unclear, although it appears to have an immune-mediated basis. Idiopathic, systemic disorder-related, and rarely drug-induced forms of LABD have been described. We describe a case of LABD associated with interferon-, 2A used for the treatment of Kaposi's sarcoma. [source] Linear IgA bullous dermatosis in a patient with advanced pancreatic carcinomaCLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 4 2008M. Adami No abstract is available for this article. [source] | ||||||||||