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Lymphocytotoxic autoantibodies in pemphigus and systemic lupus erythematosus.

Lymphocytotoxic autoantibody (LCTA) was studied in 9 patients with active pemphigus and 19 patients with active systemic lupus erythematosus (SLE). All patients with SLE showed high titres of LCTA but only one patient with pemphigus had LCTA. In this paper we discuss the immunological significance of LCTA and the difference in the incidence of LCTA between pemphigus and SLE. In addition we present the clinical features of the case with LCTA-positive pemphigus and suggests that the presence of LCTA is due to the common viral infection.

Antilymphocyte Serum↗

[Perioperative management of a patient with systemic lupus erythematosus, myasthenia gravis, and pemphigus foliaceous].

A 38-year-old female with systemic lupus erythematosus (SLE), myasthenia gravis (MG), and pemphigus foliaceous (PF) was scheduled to undergo total hysterectomy and lymphadenectomy. Preanesthetic examination revealed anemia, a prolonged activated partial thromboplastin time, and a reduced percent vital capacity. Antiphospholipid antibody was not positive. After treating the bullous lesions of PF and the muscle weakness due to MG (noted on admission for surgery) with oral prednisolone, the patient was scheduled for surgery. To avoid the use of a muscle relaxant and the potential complications of the airway manipulation involved in using a laryngeal mask or endotracheal tube, since the patient had MG and PF, a regional anesthetic technique was selected. This involved continuous epidural anesthesia, achieved using 1% or 2% mepivacaine, with sedation by a combination of propofol infusion (3 mg.kg-1.hr-1) and nitrous oxide (60% in oxygen). The patient breathed spontaneously under the mask throughout the 3.5-hr operation. The intraoperative surgical and anesthetic course was uneventful. After a benign postoperative course, the patient was discharged on the 16th postoperative day.

Adult↗

Coexistence of pemphigus herpetiformis and systemic lupus erythematosus.

A female patient with coexistence of pemphigus herpetiformis and systemic lupus erythematosus is described. She presented to our Department with pruritic vesicles on her trunk and extremities, which were later accompanied with butterfly like erythema on her face and with central nervous system (CNS) manifestations. The diagnosis of pemphigus herpetiformis was based on the clinical picture and immunofluorescence finding, because the histopathologic finding is not always typical for the diagnosis. The diagnosis of systemic lupus erythematosus was based on positive ANA and anti-dsDNA, presence of butterfly-like erythema on her face, and CNS manifestations. The patient was treated by corticosteroids in combination with immunosuppressants, which should ensure good control of both diseases. The coexistence of pemphigus herpetiformis and systemic lupus erythematosus has not been reported in recent literature.

Abdomen↗

Graft-versus-host-like mucocutaneous eruptions with serological features of paraneoplastic pemphigus and systemic lupus erythematosus in a patient with non-Hodgkin's lymphoma.

A 63-year-old male patient spontaneously developed severe erosive orogenital mucositis, palmoplantar and gluteal inflammatory lesions resistant to therapy. The skin lesions clinically and histologically resembled lichen-planus-like graft-versus-host disease. Investigation for an underlying autoimmune or malignant disorder revealed a centrocytic-centroblastic low-grade non-Hodgkin's lymphoma (according to the Kiel classification) in the bone marrow, mesenterial and iliacal lymphoma. Serological titers were intermittently positive for ANA, anti-Sm/U1RNP, anti-Ro and anti-dsDNA. Immunoprecipitation of lysates from radiolabeled human keratinocytes with the patient's serum revealed circulating antibodies against 210-kD (desmoplakin II), 190- and 170-kD antigens but none against the 230-kD antigen or 250-kD desmoplakin I. Under cytostatic chemotherapy the lymphomas showed complete and long-lasting remission, whereas the mucocutaneous lesions persisted. Six years after diagnosis, the mucocutaneous lesions are sufficiently controlled by immunosuppressive therapy. In the presented case, several features of lymphoma-associated dysimmunoreactivity are assumed that bring about the intrinsic production of various autoantibodies typical of paraneoplastic pemphigus and systemic lupus erythematosus.

Diagnosis, Differential↗

Immunologic response to A and B erythrocytic antigen.

Immunologic response to A and B erythrocytic antigen stimulation was studied in patients with pemphigus, in patients with systemic lupus erythematosus (SLE), and in normal subjects. Patients with pemphigus and normal subjects demonstrated a similar specific response (isohemagglutinins). A comparison between patients with pemphigus and SLE showed higher titers in the latter. Intercellular antibody titers increased after stimulation; corresponding titers were unaffected by isohemagglutinin absorption. Direct immunofluorescent studies showed IgG deposits in the intercellular spaces of the skin of the patients with pemphigus. However, C3 was found in the same sites in only three cases of pemphigus erythematosus. Immunofluorescent studies for IgG at the basal membrane were positive in only two cases of pemphigus erythematosus. Immunologic response of patients with pemphigus was similar to that of controls; however, it differed from that of patients with SLE.

ABO Blood-Group System↗

Pemphigus vulgaris and systemic lupus erythematosus in a 46-y-old man.

Systemic lupus erythematosus (SLE) is an autoimmune disease that may affect many organs in the body. Skin manifestations are frequent and sometimes vesiculobullous lesions may apper such as in bullous lupus erythematosus. SLE may also be exceptionally associated with other blistering diseases such as pemphigus vulgaris (PV). We describe the case of a male diagnosed as having SLE, who one year later developed PV. Only three cases (all in women) of SLE associated with PV have been reported in the literature. Our case is noteworthy in that this exceptional association developed in a middle-aged male.

Humans↗

Immunofluorescence of biopsy specimens: comparison of methods of transportation.

Immunofluorescence findings for IgG, IgA, IgM, and C4 on biopsy specimens quick frozen and transported frozen were compared with findings on portions of the same biopsy specimens placed in holding solution and transported at ambient temperatures. A total of 52 biopsy specimens were examined from normal individuals and patients with systemic lupus erythematosus (SLE), discoid lupus erythematosus (DLE), pemphigus, pemphigoid, and dermatitis herpetiformis. Overall agreement of results was 90%: 88% in SLE; 50% to 66% in DLE, 96% in pemphigus; 92% in bullous pemphigoid; and 87% in dermatitis herpetiformis. Except for two of the 42 biopsy specimens, the combined IgG, IgA, IgM, and C4 findings were the same. In one DLE case, only the frozen biopsy specimen was positive. In one case of dermatitis herpetiformis, only the ambient temperature biopsy specimen was positive. Results indicate the holding solution at ambient temperature can be used in place of the frozen method.

Biopsy↗

[The immunomodulating action of interferon preparations. The effect of medicinal alpha-interferon preparations on the rosette-forming activity of human blood lymphocytes].

Effects of alpha-interferon (alpha-IF) medical preparations on human blood lymphocyte rosette-forming activity was under study. Blood samples from 40 donors and 81 patients with chronic pyoderma, lupus erythematosus, and pemphigus were examined. Interferon preparations alpha-IF for nasal administration and IN-1 and IN-2 (or leukinterferon) for injections were used. The results evidence that IF preparations activities depend on the methods of the preparation synthesis and purification. alpha-IF and leukinterferon most effectively enhanced the lymphocyte ability to spontaneous, early, and active rosette formation. IN-1 was found less active and enhanced only the ability to spontaneous E-rosette formation, but had no effect on early active E-RFC subpopulations. IF preparations elevated the counts of E-RFC anf active RFC only if these levels were lowered; if these values were normal the agents had no effect.

Adjuvants, Immunologic↗