1/13. BP180 ELISA using bacterial recombinant NC16a protein as a diagnostic and monitoring tool for bullous pemphigoid.Bullous pemphigoid (BP) is an acquired autoimmune subepidermal blistering disease against hemidesmosomal cytoplasmic BP230 and transmembrane BP180 proteins. epitope mapping studies have shown that the membrane-proximal noncollagenous (NC) 16a domain of BP180 harbors clusters of antigenic sites recognized by the vast majority of BP sera. In this study, we developed an enzyme-linked immunosorbent assay (ELISA) using bacterial recombinant NC16a protein and evaluated its clinical benefit for diagnosis and monitoring disease activity. Fifty four (84.4%) of 64 sera from BP patients were positive, while only one (1.1%) of 91 sera from collagen disease patients and five (1.5%) of 336 sera from normal control barely exceeded the cut-off value. None of 69 pemphigus vulgaris sera and none of 42 pemphigus foliaceus sera exceeded the cut-off value. Thus, the sensitivity and specificity of NC16a ELISA were 84.4 and 98.9%, respectively. The correlation between ELISA scores and disease activity along the time course was examined using seven BP patients. NC16a ELISA scores tended to fluctuate in parallel with the disease activity along the time course and reflected the disease activity much better than indirect immunofluorescence. These findings indicate that NC16a ELISA will be a valuable tool not only for the diagnosis of patients with BP but also for the monitoring of the disease activity.- - - - - - - - - - ranking = 1keywords = vulgaris (Clic here for more details about this article) |
2/13. Acantholytic cells exfoliated from pemphigus vulgaris of the uterine cervix. A case report.BACKGROUND: pemphigus vulgaris of the uterine cervix is rare and almost always associated with cutaneous or mucosal lesions elsewhere on the body. Without a history of pemphigus, acantholytic cells in cervical smears may be misdiagnosed as malignant ones. CASE: A 52-year-old female presented with a vaginal discharge, and a routine cervical smear was collected for cytology. The smear displayed atypical cells lying singly and in loose clusters, having vesicular nuclei, a thin nuclear membrane, prominent nucleoli and well-defined cytoplasmic margins. These were labeled atypical glandular cells of undetermined significance, and colposcopic examination and biopsy were advised. On colposcopy vesicular lesions and erosions were noticed on the cervix. The biopsy revealed typical intact as well as denuded suprabasal bullae of pemphigus vulgaris. On reevaluation of the cytologic smear, the cytomorphologic features correlated well with the acantholytic cells of pemphigus. Thorough reexamination of the patient revealed 2 tiny vesicles on the oral mucosa that, on biopsy, confirmed the diagnosis of pemphigus vulgaris. CONCLUSION: Cytopathologists should be aware of the typical cytomorphologic features of pemphigus vulgaris and, in an appropriate clinical setting, should be able to diagnose or at least suspect this entity in even rare sites like the cervix. A false positive diagnosis of malignancy can be avoided if the cytologic findings are judiciously correlated with the history and with clinical and colposcopic examinations.- - - - - - - - - - ranking = 8keywords = vulgaris (Clic here for more details about this article) |
3/13. Bullous pemphigoid and psoriasis vulgaris.A 55-year-old man with psoriasis presented with erythematous, edematous plaques, tense bullae, and erosions. Direct immunofluorescence and histopathologic examination confirmed the diagnosis of bullous pemphigoid. Both dermatologic conditions responded to azathioprine. The clinical presentation, etiology, and treatment options are reviewed.- - - - - - - - - - ranking = 4keywords = vulgaris (Clic here for more details about this article) |
4/13. Two cases of subepidermal blistering disease with anti-p200 or 180-kD bullous pemphigoid antigen associated with psoriasis.We describe two patients with psoriasis vulgaris who subsequently developed a subepidermal blistering disease which disclosed IgG and C3 at the basement membrane zone in direct immunofluorescence. The first case was a 75-year-old Japanese man with herpetiform lesions. Histopathology showed neutrophil infiltration. IgG antibodies bound to the dermal side of the salt-split skin. Immunoblot analysis identified a 200-kD antigen in dermal extracts. The second case was a 70-year-old Japanese man. Histopathology showed eosinophil infiltration. IgG antibodies bound to the epidermal side of salt-split skin. Immunoblot analysis identified a 180-kD bullous pemphigoid (BP) antigen in epidermal extracts. We review the clinical and pathological features of psoriatic patients who presented a subepidermal blistering disease in which antigens were detected by immunoblot analysis; i.e., anti-p200 pemphigoid (14 cases) or BP (12 cases). There were few distinct clinical differences between two diseases. However, neutrophils were predominant in anti-p200 pemphigoid, while eosinophils were predominant in BP. After blister formation, ciclosporin was used effectively in addition to systemic steroids in the treatment of anti-p200 pemphigoid. On the other hand, ciclosporin was not used in the treatment of BP with psoriasis.- - - - - - - - - - ranking = 1keywords = vulgaris (Clic here for more details about this article) |
5/13. Coexistence of pemphigus vulgaris and bullous pemphigoid in the upper aerodigestive tract.pemphigus vulgaris and bullous pemphigoid are autoimmune blistering diseases of the skin and the mucosa characterized by circulating autoantibodies. Coexistence of these lesions is extremely uncommon. We report herein a case of both pemphigus vulgaris and bullous pemphigoid which occurred in the upper aerodigestive tract. The diagnosis was made based on the circulating autoantibodies and direct immunofluorescent studies. The literature on this subject is reviewed.- - - - - - - - - - ranking = 6keywords = vulgaris (Clic here for more details about this article) |
6/13. 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 = vulgaris (Clic here for more details about this article) |
7/13. 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) |
8/13. 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) |
9/13. A case of bullous pemphigoid with antibodies against intercellular 130 kd antigen.pemphigus and bullous pemphigoid are two typical autoimmune bullous diseases that involve circulating autoantibodies directed against the epidermal cell surface and the epidermal basement membrane zone, respectively. The coexistence of pemphigus and bullous pemphigoid is rare. We describe a case of a 79-year-old man who had tense bullae and erythematous, erosive lesions on his trunk and four extremities. Histopathology revealed subepidermal blister formation without any evidence of intraepidermal acantholytic changes. Direct immunofluorescence study demonstrated deposition of IgG on the epidermal intercellular spaces, as well as along the basement membrane zone; C3 was detected only on the latter. Indirect immunofluorescence study using monkey esophagus as a substrate demonstrated the presence of circulating antibodies against both junctional and intercellular antigens. In order to analyze the precise nature of this patient's antibodies, indirect immunofluorescence study using cultured human keratinocytes and immunoblot analyses were performed. pemphigus vulgaris sera showed smooth and uniform staining on intercellular spaces. The patient's serum showed a granular and uneven staining pattern. Immunoblot analysis showed that the patient's serum reacted with the typical 230 kd (bullous pemphigoid) antigen and 130 kd antigen, which is close to the pemphigus vulgaris antigen.- - - - - - - - - - ranking = 2keywords = vulgaris (Clic here for more details about this article) |
10/13. Bullous pemphigoid complicated with pemphigus vulgaris?A 67-year-old housewife had polymorphous eruptions with tense bullae on the extremities and upper trunk. Erosions were noted on the oral and esophageal mucosa. A skin biopsy showed subepidermal blistering with linear deposition of IgG and C3 at the basement membrane zone. In addition, intercellular deposition was observed at the lower part of the epidermis. On the basis of clinical and histological features, a diagnosis of bullous pemphigoid (BP) was made. After she was treated with prednisolone, the whole-skin lesions soon disappeared. An esophageal biopsy showed intercellular deposition of IgG and C3. Indirect immunofluorescence testing using esophageal mucosa of guinea pigs showed the same deposition at the titer of 1:20. Immunoblottings with this patient's serum revealed no reactions with either BP or pemphigus antigens. Her serum reacted strongly with a 190-kD protein, the nature of which was unknown. A treatment with ciclosporin was added to this steroid therapy, and the mucosal lesions improved gradually.- - - - - - - - - - ranking = 4keywords = vulgaris (Clic here for more details about this article) |
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