1/329. Childhood pemphigus.BACKGROUND: Five children with pemphigus are reported: three with pemphigus vulgaris, one with pemphigus vegetans, and one with pemphigus foliaceus. Only one case of juvenile pemphigus vegetans has been published in the literature. MATERIALS AND methods: All three patients with pemphigus vulgaris were treated with oral corticosteroid; in two cases, azathioprine was added for steroid-sparing effect. The patient with pemphigus vegetans had a clinical presentation resembling pemphigus vulgaris, but the lesions in the perianal area healed as hypertrophic granulation tissue. He was treated with oral corticosteroid, azathioprine, and intralesional corticosteroid. The patient with pemphigus foliaceus presented with exfoliative dermatitis, and was treated with oral corticosteroid; methotrexate was added later for steroid-sparing purposes RESULTS: The patients were followed up for 1-4 years; the prognosis of childhood pemphigus is good. CONCLUSIONS: Long-term follow-up is needed to detect flaring of the disease and the side-effects of immunosuppressive drugs.- - - - - - - - - - ranking = 1keywords = vulgaris (Clic here for more details about this article) |
2/329. Scrape cytology of oral pemphigus. Report of a case with immunocytochemistry and light, scanning electron and transmission electron microscopy.BACKGROUND: pemphigus vulgaris is a disseminated disease of the skin and mucous membranes characterized by recurrent vesicular and bullous lesions due to the autoantigen belonging to the cadherin type of cell adhesion molecules. The presence of acantholysis associated with immunoglobulins in the intercellular spaces and on the cell membrane are diagnostic features. However, the appearance of smears from the oral cavity by scanning (SEM) and transmission electron microscopic (TEM) study as well as immunocytochemistry of cadherin does not appear to have been previously reported. CASE: A 67-year-old female developed erosion on her gingiva with severe pain. On oral examination, there were ulcerations on the palate, and the Nikolsky sign was positive. The characteristic cytologic findings from oral scrapes were high cellularity, a bloody background and a predominant cell population consisting of polygonal basal and parabasal cells with pronounced nucleoli. Also present were degenerative cell changes: e.g., cytoplasmic vacuoles and a homogeneous nuclear appearance. Immunocytochemical staining for IgG and cadherin gave a positive reaction in the intercellular spaces and on the cell membranes. The surface of cells in pemphigus vulgaris by SEM showed somewhat irregularly distributed microridges, and TEM revealed desmosomal attachments, degenerated tonofilaments with pronounced nucleoli and heterochromatin. As a result of cytodiagnosis, additional appropriate specimens were obtained at the time of the scraping for confirmatory immunocytochemistry for cadherin, SEM and TEM studies. CONCLUSION: The results demonstrate that a precise diagnosis of pemphigus vulgaris can be rendered on cellular material and cadherin immunocytochemistry obtained by scrape from the oral mucosa.- - - - - - - - - - ranking = 1keywords = vulgaris (Clic here for more details about this article) |
3/329. mortality associated with concurrent strongyloidosis and cytomegalovirus infection in a patient on steroid therapy.Disseminated strongyloidosis has been recognized with increasing frequency, often in patients who are immunocompromised or have received steroid therapy. In addition, disease due to cytomegalovirus (CMV) is noted in immunodeficient hosts. We report on a 55-year-old Puerto Rican man who received steroid treatment for orpharyngeal pemphigus vulgaris and developed abdominal symptoms with alternating constipation and diarrhea. The clinical work-up did not reveal specific abnormalities, but the patient died of cardiopulmonary failure. At the postmortem examination, the patient had evidence of strongyloidosis and CMV disease. This report reviews both this case and the literature, and discusses the overlapping infections of strongyloidosis and CMV disease in this patient who had received steroid therapy.- - - - - - - - - - ranking = 0.33333333333333keywords = vulgaris (Clic here for more details about this article) |
4/329. esophagitis dissecans superficialis associated with pemphigus vulgaris.The extension of bullous lesions in pemphigus to the esophagus is relatively uncommon, especially in patients who appear to be in clinical remission. Very rarely, pemphigus vulgaris may affect the entire esophagus, resulting in complete sloughing of the mucous membrane. A 20-year-old man with pemphigus vulgaris presented to the emergency room with acute onset of dysphagia, odynophagia, and hemoptysis. There were no cutaneous or oral findings of pemphigus on presentation, since he was being maintained on corticosteroids and azathioprine with excellent results. During initial evaluation in the emergency room, the patient was observed to vomit a cast of the mucosal lining of the esophagus. The morphologic description of such an esophageal cast is termed esophagitis dissecans superficialis. This is the third case of esophagitis dissecans superficialis in pemphigus vulgaris recorded in the medical literature.- - - - - - - - - - ranking = 2.3333333333333keywords = vulgaris (Clic here for more details about this article) |
5/329. pemphigus vulgaris preceded by herpetiform-like skin lesions with negative immunofluorescence findings.We report a case of pemphigus vulgaris in a 63-year-old woman. At the first onset, she developed herpetiform-like skin lesions. Their histology showed eosinophilic spongiosis and intraepidermal blister formation with an infiltrate of eosinophils. Both direct and indirect immunofluorescence studies were negative. She remained in remission for 6 years. At the second onset, she developed bullous skin lesions; histological examination disclosed suprabasal acantholysis and infiltration of eosinophils. Direct immunofluorescence revealed IgG and C3 deposits at the cell surface, predominantly in the lower epidermis. Indirect immunofluorescence using normal human skin as a substrate demonstrated anti-cell surface IgG antibodies at a titer of 1:160. Immunoblot analysis showed that the patient's serum reacted only with a 130 kD protein. This case indicates that pemphigus vulgaris can follow herpetiform-like skin lesions with negative immunofluorescence findings after a long remission time.- - - - - - - - - - ranking = 2keywords = vulgaris (Clic here for more details about this article) |
6/329. 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 = 0.33333333333333keywords = vulgaris (Clic here for more details about this article) |
7/329. pemphigus vulgaris in pregnancy with favourable foetal prognosis.pemphigus vulgaris is an immunobullous disease affecting the skin and mucous membranes most commonly during the fifth and sixth decades of life. Its occurrence in pregnancy is rare. We now report two severe cases of the disorder presenting during pregnancy and discuss its potential effects on the foetus and its management in pregnancy.- - - - - - - - - - ranking = 1.6666666666667keywords = vulgaris (Clic here for more details about this article) |
8/329. Successful treatment of adolescent pemphigus vulgaris by immunoadsorption method.A 15-year-old girl with pemphigus vulgaris did not respond to oral administration of prednisolone at 45 mg/day. The skin and oral mucous membrane lesions recurred after effective treatments with methylprednisolone pulse therapy and combination therapy with prednisolone and cyclosporine. The finally successful treatment involved eleven cycles of immunoadsorption using a tryptophan column and administration of a moderate dose of prednisolone. serum gamma-globulin level and anti-intercellular antibody titer decreased from 1.08 g/dl to 0.5 g/dl and 1:320 to 1:20, respectively. She has been well controlled with 21.5 mg/day prednisolone for 8 months after the final adsorption. Considering the physical, mental and social situation of adolescent student patients, immunoadsorption is a highly preferable choice among a variety of treatment modalities for pemphigus vulgaris because it makes the term of hospitalization shorter and avoids undesirable side effects from initial high dose corticosteroids.- - - - - - - - - - ranking = 2keywords = vulgaris (Clic here for more details about this article) |
9/329. pemphigus vulgaris localized to the vagina presenting as chronic vaginal discharge.Involvement in pemphigus vulgaris of the female genital tract including the vulva, vagina and cervix has previously been described. In all these cases other cutaneous and mucosal sites have also been affected at some time. We describe a case of pemphigus vulgaris which only involved the vaginal mucosa. The patient presented with a persistent vaginal discharge and examination showed extensive vaginal erosions. histology of vaginal biopsies was non-diagnostic. The recognition that the vaginal changes may represent an immunobullous disease led to further vaginal biopsies on which direct immunofluorescence studies were performed. These biopsies showed IgG and C3 in the intercellular epidermis, suggesting a diagnosis of pemphigus vulgaris. During the 3-year period that the patient has been under review there have never been any other cutaneous or mucosal lesions. To our knowledge, this is the first case of pemphigus vulgaris localized exclusively to the vaginal mucosa. There was considerable delay in diagnosis and this case highlights how important it is to recognize that chronic mucosal lesions at genital sites may be caused by immunobullous diseases such as cicatricial pemphigoid and pemphigus, and to institute appropriate investigations.- - - - - - - - - - ranking = 2.6666666666667keywords = vulgaris (Clic here for more details about this article) |
10/329. Mycophenolate mofetil: a new therapeutic option in the treatment of blistering autoimmune diseases.BACKGROUND: Mycophenolate mofetil (MMF), an ester of mycophenolic acid (MPA), was approved by the food and Drug Administration in 1995 and is currently primarily indicated for the prophylaxis of rejection in renal transplant patients. The drug seems also to be of value in the treatment of psoriasis and rheumatic arthritis. Recently there have been 6 reported cases of successful treatment of blistering autoimmune diseases with MMF in combination with high dose prednisone therapy. OBJECTIVE: On the basis of these reports we administered this new treatment regimen to several patients with blistering autoimmune diseases. Besides using a combination of MMF and high-dose prednisone we wanted to evaluate whether MMF monotherapy is also effective in the treatment of blistering autoimmune diseases. methods: We administered MMF to 5 patients who had severe pemphigus vulgaris or bullous pemphigoid. Two patients received MMF in combination with high-dose prednisone therapy and 3 patients received MMF monotherapy. To our knowledge, this is the first report of successful treatment of pemphigus vulgaris and bullous pemphigoid with MMF monotherapy. RESULTS: All patients were completely free of symptoms within 8 to 11 weeks of therapy. patients who had received MMF monotherapy responded as well to treatment as those who received a combination of MMF and high-dose prednisone. CONCLUSION: Our experiences strongly suggest that MMF monotherapy may be effective for patients even with severe pemphigus vulgaris and bullous pemphigoid. In addition, MMF monotherapy, at least over the short term, offers the advantage of fewer side effects in comparison to immunosuppressive combination therapy and was well tolerated by our patients.- - - - - - - - - - ranking = 1keywords = vulgaris (Clic here for more details about this article) |
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