A 55-year-old White woman was first seen in July 1995 with ulceration of the oral mucosa, including the gingiva, labial mucosa, gums, palate and tongue, as well as erosions and blisters on the trunk. A biopsy showed a...A 55-year-old White woman was first seen in July 1995 with ulceration of the oral mucosa, including the gingiva, labial mucosa, gums, palate and tongue, as well as erosions and blisters on the trunk. A biopsy showed a suprabasal blister with acantholysis. Direct immunofluorescence was positive for intercellular deposits of IgG and C3, and indirect immunofluorescence was positive for intercellular antibodies at a titer of 160. The patient was diagnosed as having pemphigus vulgaris and treated with prednisone 1-1.5 mg/kg/day from 1995 until 1998, but no response was observed (the disease continued to be active with the formation of new lesions. The patent develoyd a number of steroid-complications including diabetes, osteoporosis, Cushing syndrome, hypertension and high-output cardiac failure, and required repeated hospitalization. In August 1998 she was started on azathioprine 100-150 mg/day and continued on prednisone 1 mg/kg/day. One year later, in August 1999, the disease was still active with new lesion formation and persistent oral erosions and dysphagia, despite persistent therapywith azathioprine 150 mg/day and prednisone 1mg/kg/day. Because of the disease severity and the lack of a reponse to treatment, in October 1999 the patient was started on thalidomide therapy 100 mg/day (1.7 mg/kg/day) and continued the prednisone treatment at 1 mg/kg /day together with azathioprine 150 mg/day (2.7 mg/kg/day). There was a good response, with clearing of all oral lesions in 20 days. The prednisone dose was gradually reduced and was discontinued in May 2000; azathioprine was gradually reduced to 50 mg 3 times/week (0.35 mg/kg/day). Thalidomide was continued at a dose of 100 mg/day. The patient was in total clinical remission, being free of both old and new lesions, for the next 14 months, when the thalidomide treatmentwas discontinued as a result of side-effects, including weakness in the legs and paresthesis of the fingers. After the discontinuance of treatment with thalidomide, severe lesions returned. Thalidomide was reintroduced, at 100 mg/day. After 2 months the patient entered total clinical remission and on the last occasion on which she was seen, in May 2003, was found to have remained clear of lesions. The patient continues to take thalidomide at 100 mg/day.展开更多
Background: Since 1967 dermatology has used the classic technique of indirect immunofluorescence (IIF) for the detection of autoantibodies against antigens of the skin in diseased people with endemic pemphigus foliace...Background: Since 1967 dermatology has used the classic technique of indirect immunofluorescence (IIF) for the detection of autoantibodies against antigens of the skin in diseased people with endemic pemphigus foliaceus. Thirty years later enzyme-linked immunosorbent assays -ELISA (rDsg1 and rDsg3) appeared as a viable option. A group of highly recognized researchers have concluded that ELISA is a simple, sensitive and highly specific method, allowing for diagnostic differentiation between pemphigus vulgaris (PV) and endemic pemphigus foliaceus (EPF). Scientific literature certifies that both ELISA and IIF bear high sensitivity in spite of the fact that a direct comparison between the ELISA and IIF tests has never been performed. Objectives: This study was conducted to compare the sensitivity of these tests in detecting antibodies in the EPF. Material and methods: Thirty-two serum samples were collected from patients with EPF. The control serum of 15 healthy individuals was tested to detect the presence of antibodies of EPF by indirect immunofluorescence and ELISA (rDsg1 and rDsg3). The IIF was performed, taking human skin as a substrate. Results: Antibodies in patients with EPF were detected more commonly by the ELISA (rDsg1) (91%) compared with IIF (81%). Conclusions: The ELISA (rDsg1) is slightly more sensitive than IIF in detecting antibodies related to EPV. However, according to our results, we do not currently possess a test with 100%accuracy in differentiating EPF from PV. Although previous studies have associated Dsg3 with PV, the tests performed during this study showed that 12%(4/32) of patients with EPF (cutaneous diseases only) also had Dsg3 antibodies.展开更多
文摘A 55-year-old White woman was first seen in July 1995 with ulceration of the oral mucosa, including the gingiva, labial mucosa, gums, palate and tongue, as well as erosions and blisters on the trunk. A biopsy showed a suprabasal blister with acantholysis. Direct immunofluorescence was positive for intercellular deposits of IgG and C3, and indirect immunofluorescence was positive for intercellular antibodies at a titer of 160. The patient was diagnosed as having pemphigus vulgaris and treated with prednisone 1-1.5 mg/kg/day from 1995 until 1998, but no response was observed (the disease continued to be active with the formation of new lesions. The patent develoyd a number of steroid-complications including diabetes, osteoporosis, Cushing syndrome, hypertension and high-output cardiac failure, and required repeated hospitalization. In August 1998 she was started on azathioprine 100-150 mg/day and continued on prednisone 1 mg/kg/day. One year later, in August 1999, the disease was still active with new lesion formation and persistent oral erosions and dysphagia, despite persistent therapywith azathioprine 150 mg/day and prednisone 1mg/kg/day. Because of the disease severity and the lack of a reponse to treatment, in October 1999 the patient was started on thalidomide therapy 100 mg/day (1.7 mg/kg/day) and continued the prednisone treatment at 1 mg/kg /day together with azathioprine 150 mg/day (2.7 mg/kg/day). There was a good response, with clearing of all oral lesions in 20 days. The prednisone dose was gradually reduced and was discontinued in May 2000; azathioprine was gradually reduced to 50 mg 3 times/week (0.35 mg/kg/day). Thalidomide was continued at a dose of 100 mg/day. The patient was in total clinical remission, being free of both old and new lesions, for the next 14 months, when the thalidomide treatmentwas discontinued as a result of side-effects, including weakness in the legs and paresthesis of the fingers. After the discontinuance of treatment with thalidomide, severe lesions returned. Thalidomide was reintroduced, at 100 mg/day. After 2 months the patient entered total clinical remission and on the last occasion on which she was seen, in May 2003, was found to have remained clear of lesions. The patient continues to take thalidomide at 100 mg/day.
文摘Background: Since 1967 dermatology has used the classic technique of indirect immunofluorescence (IIF) for the detection of autoantibodies against antigens of the skin in diseased people with endemic pemphigus foliaceus. Thirty years later enzyme-linked immunosorbent assays -ELISA (rDsg1 and rDsg3) appeared as a viable option. A group of highly recognized researchers have concluded that ELISA is a simple, sensitive and highly specific method, allowing for diagnostic differentiation between pemphigus vulgaris (PV) and endemic pemphigus foliaceus (EPF). Scientific literature certifies that both ELISA and IIF bear high sensitivity in spite of the fact that a direct comparison between the ELISA and IIF tests has never been performed. Objectives: This study was conducted to compare the sensitivity of these tests in detecting antibodies in the EPF. Material and methods: Thirty-two serum samples were collected from patients with EPF. The control serum of 15 healthy individuals was tested to detect the presence of antibodies of EPF by indirect immunofluorescence and ELISA (rDsg1 and rDsg3). The IIF was performed, taking human skin as a substrate. Results: Antibodies in patients with EPF were detected more commonly by the ELISA (rDsg1) (91%) compared with IIF (81%). Conclusions: The ELISA (rDsg1) is slightly more sensitive than IIF in detecting antibodies related to EPV. However, according to our results, we do not currently possess a test with 100%accuracy in differentiating EPF from PV. Although previous studies have associated Dsg3 with PV, the tests performed during this study showed that 12%(4/32) of patients with EPF (cutaneous diseases only) also had Dsg3 antibodies.
