1/14. Resolution of bullous pemphigoid and improvement of vitiligo after successful treatment of squamous cell carcinoma of the skin.The significance of the association of malignant diseases with bullous pemphigoid is still unknown. We report a case of squamous cell carcinoma of the skin associated with both bullous pemphigoid and vitiligo. It is possible that there is a common underlying pathogenic mechanism involved in the co-existence of these three skin diseases as successful treatment of the carcinoma was accompanied by resolution of the bullous pemphigoid and improvement of the vitiligo.- - - - - - - - - - ranking = 1keywords = skin disease (Clic here for more details about this article) |
2/14. epidermolysis bullosa acquisita with combined features of bullous pemphigoid and cicatricial pemphigoid.epidermolysis bullosa acquisita (EBA) is an acquired subepidermal blistering disease associated with autoantibodies against type VII collagen. The classical or mechanobullous form of EBA is characterized by skin fragility, trauma-induced blisters and erosions with mild mucous membrane involvement and healing with scars. Furthermore, bullous-pemphigoid-like and cicatricial pemphigoid-like features have been described. We report a patient who developed a bullous skin disease with upper airway obstruction requiring tracheotomy. The diagnosis of EBA was established by immunoblot, showing a band at 290 kD (collagen VII), and NaCl-split skin immunofluorescence (IgG deposition at the floor of the split). This case presented with clinical features of both bullous pemphigoid and cicatricial pemphigoid which to our knowledge is the first report of such a combination in EBA. The patient also presented tracheal involvement that has never been described either.- - - - - - - - - - ranking = 1keywords = skin disease (Clic here for more details about this article) |
3/14. lichen planus pemphigoides with IgG autoantibodies to the 180 kd bullous pemphigoid antigen (type XVII collagen).We describe a 75-year-old patient with pruritic papules on her trunk and extremities, typical of lichen planus, who later experienced subepidermal blisters. These clinical features are consistent with lichen planus pemphigoides. Immunofluorescence of perilesional skin showed linear deposits of C3 along the dermoepidermal junction. Circulating IgG autoantibodies were found to be directed against an epidermal component of the dermoepidermal junction because the patient's serum labeled the epidermal side of 1 mol/L NaCl-split skin. The patient's IgG autoantibodies were directed exclusively against the 180 kd bullous pemphigoid antigen (BPAg2, type XVII collagen) detected in human keratinocyte lysate by Western blot assay. No reactivity was found against the 230 kd bullous pemphigoid antigen, type VII collagen, or the laminin-5 subunits. This study demonstrates that BPAg2 is recognized, not only by bullous pemphigoid sera, but also by lichen planus pemphigoides sera. Our findings attest to the similarity of immunopathology in these two subepidermal blistering skin diseases.- - - - - - - - - - ranking = 1keywords = skin disease (Clic here for more details about this article) |
4/14. Bullous pemphigoid in a leg affected with hemiparesia: a possible relation of neurological diseases with bullous pemphigoid?We report a typical case of bullous pemphigoid (BP) associated with a neurological disorder and study a possible link between neurological disorders and BP. An 84-year-old hemiplegic woman presented with unilateral BP on the hemiparetic side. BP was confirmed by histological and immunofluorescence data. The medical records of the previous 46 consecutive patients with BP were retrospectively analyzed (average age: 79; median age: 85). Thirty of the 46 patients with BP had neurological disorders. These disorders included dementia, epilepsy, multiple sclerosis, cerebral stroke, Parkinson's disease, gonadotropic adenoma, trembling, dyskinesia, lumbar spinal stenosis. In a control group of the 46 consecutive oldest patients (older than 71; average age: 82,5; median age: 80) with another skin disease referred during the previous two-year-period to our one-day-unit only, 13 patients had a neurological disorder. This study demonstrates that there is a high prevalence of neurological disorders in patients with BP (p = 0.0004). A prospective case control study with neurological examination and psychometrical evaluation is warranted to confirm these data. We speculate that neuroautoimmunity associated with the aging process or neurological disorders may be involved in pemphigoid development via an autoimmune response against dystonin which shares homology with bullous pemphigoid antigen 1. Bullous pemphigoid could be considered to be a marker of neurological disorder.- - - - - - - - - - ranking = 1keywords = skin disease (Clic here for more details about this article) |
5/14. Bullous pemphigoid in a patient with psoriasis and possible drug reaction: a case report.Bullous pemphigoid, first described in 1953 by Lever, is the most common autoimmune blistering skin disease. As such,this disorder may be encountered relatively frequently in the dermatologist's office, though it is a rarity on the inpatient medicine wards. Lesions develop when autoantibodies are directed against glycoproteins BPAG1 and BPAG2, hemidesmosomes that are embedded in the epidermal basement membrane. The immune complex triggers a complement cascade that activates neutrophils and eosinophils, resulting in the release of proteolytic enzymes. As a result, subepidermal bullae develop; the diagnosis is confirmed by histological examination of skin biopsy. The case of bullous pemphigoid described here presented the treating team with some diagnostic challenges because of the patient's concomitant history of psoriasis and apparent dermatologic reaction to cephalexin. Though the patient's skin lesions resolved rapidly with appropriate treatment, the case generated many questions about the diagnosis and most effective management of bullous pemphigoid.- - - - - - - - - - ranking = 1keywords = skin disease (Clic here for more details about this article) |
6/14. Subepidermal blistering disease with autoantibodies to both the p200 autoantigen and the alpha3 chain of laminin 5.Anti-p200 pemphigoid and anti-laminin 5 mucous membrane pemphigoid are two distinct autoimmune blistering skin diseases. patients with anti-p200 pemphigoid demonstrate circulating autoantibodies to an unknown 200-kd acidic noncollagenous glycoprotein of the lower lamina lucida, whereas anti-laminin 5 mucous membrane pemphigoid is characterized by an autoimmune response against the major basement membrane adhesion molecule laminin 5. In this report, we describe a patient who developed a recurrent nonscarring blistering eruption, affecting both skin and mucous membranes. immunoblotting of dermal extracts and extracellular matrix of cultured keratinocytes revealed circulating autoantibodies directed to the p200 antigen and the alpha3 chain of laminin 5, respectively. The unusual clinical and immunologic profile of this case suggests that molecular specificity of circulating autoantibodies affects the clinical presentation of autoimmune subepidermal blistering skin diseases.- - - - - - - - - - ranking = 2keywords = skin disease (Clic here for more details about this article) |
7/14. Successful adjuvant treatment of severe bullous pemphigoid by tryptophan immunoadsorption.Bullous pemphigoid (BP) is an autoimmune blistering skin disease associated with circulating autoantibodies to the hemidesmosomal antigens BP180 and BP230. We report two cases of therapy-refractory BP adjuvantly treated by tryptophan immunoadsorption. In both patients, this treatment was associated with rapid clinical improvement and reduction in the required corticosteroid dosage. In addition, levels of circulating anti-BP180 autoantibodies decreased markedly. Antibodies that were eluted from the tryptophan matrix bound to BP180 and induced dermal-epidermal separation in cryosections of human skin. Our observations suggest that immunoadsorption may be a helpful adjuvant treatment in severe BP.- - - - - - - - - - ranking = 1keywords = skin disease (Clic here for more details about this article) |
8/14. Coexistence of bullous pemphigoid, vitiligo, and thyroid disease: a multiple autoimmune syndrome?Bullous pemphigoid is the most common autoimmune blistering skin diseases. The significance of the association of bullous pemphigoid with other autoimmune diseases is still unknown. There have been reports of an association with many autoimmune skin diseases. We report the simultaneous occurrence of bullous pemphigoid and thyroid disease in a 76-year-old patient who also suffered from vitiligo. It is possible that there is a common underlying pathogenic mechanism involved in the co-existence of these three diseases. The association adds to the documentation of bullous pemphigoid co-existing with other autoimmune disorders. This association is probably not fortuitous and suggests a pathogenic relationship.- - - - - - - - - - ranking = 2keywords = skin disease (Clic here for more details about this article) |
9/14. Concomitant psoriasis and bullous pemphigoid: coincidence or pathogenic relationship?psoriasis vulgaris and bullous pemphigoid represent two clinically well-characterized, chronic, inflammatory skin diseases. The concomitant occurrence of these two entities in a patient is rare, and the pathogenic implications of this phenomenon are unknown. We describe a 55-year-old woman with a 25-year history of plaque-type psoriasis who presented with disseminated tense bullae. Skin biopsies showed the typical histologic and immunohistochemical traits of bullous pemphigoid, and she had circulating immunoglobulin g (IgG) antibodies against the basement membrane zone, specifically the BP180 antigen. The bullous eruption was successfully treated with oral methylprednisolone and dapsone. Bullous pemphigoid is the autoimmune blistering disease that has most often been associated with psoriasis. Forty cases have been described in the literature. Classical psoriasis and psoriasis associated with bullous pemphigoid are identical. The pathogenic relationship between psoriasis and bullous pemphigoid is unclear. It has been postulated that the autoimmune process responsible for bullous pemphigoid lesions may be induced by ultraviolet light therapy, topical corticosteroids, and/or the inflammatory processes that occur in psoriasis. Immunomodulatory therapy may positively influence shared as well as distinct inflammatory mechanisms in patients who have psoriasis and bullous pemphigoid.- - - - - - - - - - ranking = 1keywords = skin disease (Clic here for more details about this article) |
10/14. Bullous pemphigoid-associated nephropathy: report of two cases and review of the literature.Bullous pemphigoid has previously been reported in association with a variety of renal lesions. Two additional cases are presented in this report in which the nephropathy preceded the onset of the skin disease: one case with membranous glomerulopathy and one case of renal allograft rejection with concurrent membranous pathology. Both patients had positive immunofluorescence of the skin, typical of bullous pemphigoid. Institution of systemic corticosteroid therapy resulted in a satisfactory clinical response and cessation of the blistering process. These cases and a review of the literature suggest that the occurrence of an immune process involving these two different basement membranes is not merely coincidental. Many cases have been described in which the severity of the skin lesions paralleled that of the renal disease. Although the possibility of multiple distinct autoimmune processes cannot be excluded, anti-basement-zone antibody interactions or allograft rejection-induced immune stimulation are possible unifying mechanisms for the simultaneous skin and renal involvement observed in these two cases.- - - - - - - - - - ranking = 1keywords = skin disease (Clic here for more details about this article) |
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