1/10. Three different autoimmune bullous diseases in one family: is there a common genetic base?We report an unusual familial occurrence of autoimmune bullous diseases. Three members of a family suffered from three different autoimmune bullous diseases: pemphigus vulgaris (PV), linear IgA disease (LAD) and cicatricial pemphigoid (CP). The HLA type was determined in five family members: all were positive for HLA-DQ5/DR6, which is reported to be associated with susceptibility to PV. The CP patient was DQ7(3) positive, which is in concordance with enhanced susceptibility to ocular CP and CP. The LAD patient was B8 and DR3 negative but positive for HLA-A1. Our study supports the hypothesis that there is a genetically transmitted susceptibility to autoimmune bullous diseases but that additional factors seem necessary actually to develop a particular disease.- - - - - - - - - - ranking = 1keywords = vulgaris (Clic here for more details about this article) |
2/10. staphylococcal scalded skin syndrome complicating acute generalized pustular psoriasis.A 60-year-old woman with psoriasis vulgaris treated with oral cyclosporin and acitretin developed an acute generalized pustular eruption with erythema and associated fever consistent with acute generalized pustular psoriasis. She was admitted to hospital and, despite intravenous fluid replacement, developed acute renal failure. In addition, she developed staphylococcal septicaemia. After transfer to the intensive care unit because of deteriorating renal function, a sudden onset of widespread flaccid blistering (Nikolsky sign positive) and superficial erosions was noted. histology of a biopsied blister revealed subcorneal splitting of the epidermis consistent with staphylococcal scalded skin syndrome. The patient was treated with intravenous dicloxacillin and the blistering gradually improved over 10 days.- - - - - - - - - - ranking = 1keywords = vulgaris (Clic here for more details about this article) |
3/10. UV light-induced linear IgA dermatosis.Various exogenous factors (eg, drugs, dietary antigens, trauma, infections, radiographs, and UV radiation) are known to induce or aggravate skin diseases. UV radiation in particular is known to induce or aggravate the autoimmune bullous diseases of pemphigus foliaceus, pemphigus vulgaris, and bullous pemphigoid. Its role in linear IgA dermatosis, however, is not well recognized. We report the second case of linear IgA dermatosis induced by intense sun exposure in which blistering was induced by UVA radiation. Furthermore, a review of the literature on photoinduced autoimmune bullous diseases and the wavelengths responsible for the induction of blistering is presented and several proposed mechanisms of action for the blister induction, including release or unmasking of antigens, promotion of antibody fixation by UV radiation, and launching of an inflammatory process, are discussed. We conclude that linear IgA dermatosis should be added to the list of autoimmune bullous diseases induced and/or aggravated by UV radiation.- - - - - - - - - - ranking = 1keywords = vulgaris (Clic here for more details about this article) |
4/10. Sequential use of infliximab and etanercept in generalized pustular psoriasis.Generalized pustular psoriasis is a dramatic potentially life-threatening psoriasis variant and represents a major therapeutic challenge. Tumor necrosis factor alpha (TNF-alpha) inhibitors have been shown to be highly effective in psoriasis vulgaris and psoriasis arthritis. Currently, TNF-alpha can be targeted therapeutically by 2 different approaches. TNF-alpha antibodies show a fast onset of action and a long-lasting activity. Soluble TNF-alpha receptors have a slower onset and a shorter duration of activity, which allows a rapid cessation of the drug's activity in the case of adverse events. Here we report that a remission of generalized pustular psoriasis achieved by the TNF-alpha antibody infliximab was maintained by long-term application of the soluble TNF-alpha receptor etanercept. Sequential therapy with TNF-alpha antibodies and TNF-alpha receptors may represent a novel concept that combines a rapid onset of action in the initiation therapy with a lower risk for severe adverse events in the maintenance treatment of pustular psoriasis.- - - - - - - - - - ranking = 1keywords = vulgaris (Clic here for more details about this article) |
5/10. Pitfalls in the diagnosis of pemphigus vulgaris (early pemphigus vulgaris with features of bullous pemphigoid).This report draws attention to pitfalls that may be encountered in histologic diagnosis of early stages of pemphigus vulgaris. A man is described who had clinical and histologic features of bullous pemphigoid to begin with and later typical features of pemphigus vulgaris. Intercellular antibodies were demonstrable by direct immunofluorescence from the beginning.- - - - - - - - - - ranking = 10keywords = vulgaris (Clic here for more details about this article) |
6/10. pemphigus vulgaris, an overlooked disease in young adults.Two cases of pemphigus vulgaris occurring in young men, aged 24 and 29, are presented. Prior to referral, each patient had persistent oral vesiculobullous and ulcerative mucous membrane lesions for at least six months and was treated with multiple courses of antibiotics and mouthwashes. On initial examination, both patients had the characteristic oral vesiculobullous lesions of pemphigus. A biopsy specimen of an intact vesicle confirmed the diagnosis.- - - - - - - - - - ranking = 5keywords = vulgaris (Clic here for more details about this article) |
7/10. Adjuvant high-dose intravenous gammaglobulin in the treatment of pemphigus and bullous pemphigoid: experience in six patients.At present, initial high-dose prednisone is the treatment of choice for patients with pemphigus and bullous pemphigoid. To reduce the risks associated with long-term corticosteroid treatment, other immunosuppressants are often given as steroid-sparing agents. Occasionally, the dose of steroids cannot be reduced. In this study, we report six patients with pemphigus vulgaris, pemphigus foliaceus and bullous pemphigoid, in whom the daily corticosteroid dose could only be tapered to acceptable, effective, maintenance levels following treatment with high-dose intravenous gammaglobulin.- - - - - - - - - - ranking = 1keywords = vulgaris (Clic here for more details about this article) |
8/10. A novel immune-mediated subepidermal bullous dermatosis characterized by IgG autoantibodies to a lower lamina lucida component.BACKGROUND: Immune-mediated subepidermal bullous dermatoses characterized by in vivo-bound linear IgG deposition at the cutaneous basement membrane zone include bullous pemphigoid, ocular cicatricial pemphigoid, anti-bullous pemphigoid antigen mucosal pemphigoid, anti-epiligrim mucosal pemphigoid, epidermolysis bullosa acquisita, and the bullous eruption of systemic lupus erythematosus. In this article, we describe a novel IgG-mediated bullous dermatosis. OBSERVATIONS: Clinically, a unique nonscarring dermatosis was characterized by the sudden onset of extensive bullae and erosions on mucous membrane and skin, resembling toxic epidermal necrolysis or pemphigus vulgaris. Histologically, the patient's skin lesion demonstrated neutrophilic papillary dermal infiltration and subepidermal blister formation, resembling dermatitis herpetiformis. Immunopathologically, there was linear IgG and C3 deposition at the skin basement membrane zone. The patient responded well to prednisone and azathioprine immunosuppression and has achieved a lasting remission without further therapy. Further immunologic investigations revealed that this unique dermatosis is distinct from all other known IgG-mediated subepidermal bullous dermatoses. CONCLUSIONS: This novel deep lamina lucida pemphigoid can be distinctly termed anti-p105 pemphigoid on the basis of antigenic specificity of the autoantibodies. Although this novel dermatosis resembles toxic epidermal necrolysis clinically, prudent use of diagnostic immunofluorescence studies can clearly delineate its immunologic nature. Prompt recognition of this unique dermatosis and timely initiation of appropriate immunosuppressive therapy could be life-saving for those patients suffering from this dermatosis.- - - - - - - - - - ranking = 1keywords = vulgaris (Clic here for more details about this article) |
9/10. A case of autoimmune bullous dermatosis with features of pemphigus vulgaris and bullous pemphigoid.Pleomorphic blisters, including tense bullae and annularly arranged vesicles around the erythema as well as erosive eruptions in the oral cavity, appeared on a 61-year-old woman 5 years after surgery for cholangiocellular carcinoma. A biopsy specimen from the oral cavity showed intraepidermal blisters, and those from skin lesions showed subepidermal blisters with infiltrates of eosinophils and neutrophils. The early-stage vesicles showed infiltrates along the epidermal-dermal junction, where electron microscopy disclosed disruption of the lamina densa, basal cells remaining on the dermis, and acantholytic keratinocytes among the infiltrates, but there was no cleavage of the epidermal-dermal junction at the lamina lucida. Direct immunofluorescence studies showed immune deposition at the intercellular space (ICS) and along the basement membrane zone (BMZ). Indirect immunofluorescence studies confirmed coexistence of IgG class anti-ICS and anti-BMZ antibodies. Although this case showed immunohistochemical features of bullous pemphigoid, the presence of suprabasal cleavage in the oral mucosa, acantholytic cells in the blister cavity, the deposition of IgG at the ICS of the perilesional epidermis, and circulating anti-ICS antibodies strongly suggested that this case was primarily pemphigus. The strong inflammation along the epidermal-dermal junction due to unknown factors may have modified the clinical appearance and the histopathology.- - - - - - - - - - ranking = 4keywords = vulgaris (Clic here for more details about this article) |
10/10. A case of generalized pustular psoriasis followed by bullous disease: an atypical case of bullous pemphigoid or a novel bullous disease?We describe a 31-year-old Japanese woman with generalized pustular psoriasis treated with PUVA who subsequently developed a bullous disease. Throughout the disease course, there was no phase of psoriasis vulgaris. Although several reports describe coexistence of psoriasis vulgaris and bullous disease such as bullous periphigoid, coexistence of generalized pustular psoriasis without any phase of psoriasis vulgaris and bullous disease is rare. As for the bullous disease, direct immunofluorescence study showed IgG and C3 deposition along the basement membrane zone. Indirect immunofluorescence disclosed IgG antibasement membrane zone antibodies. Indirect immunofluorescence on 1 mol/l sodium chloride-split skin demonstrated linear IgG staining almost exclusively on the dermal side of the split. Western immunoblot analysis revealed that the antibody was directed to neither epidermolysis bullosa acquisita antigen nor bullous pemphigoid antigens. Considering the unusual clinical course, we suspect the possibility of a novel autoimmune blistering disease.- - - - - - - - - - ranking = 3keywords = vulgaris (Clic here for more details about this article) |
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