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Biomedical subjects

L Engineer

Publications and source records attributed to L Engineer.

8 recordsLinked to original sources
PubMed2001

Bullous pemphigoid: interaction of interleukin 5, anti-basement membrane zone antibodies and eosinophils. A preliminary observation. .

Bullous pemphigoid (BP) is an autoimmune blistering skin disease characterized by the presence of autoantibodies to normal components of the hemidesmosome (BPAg1 and BPAg2). Histology of skin lesions demonstrate a subepidermal vesicle and a predominantly eosinophilic dermal cellular infiltrate. Interleukin 5 (IL-5) plays a major role in eosinophil recruitment and function. Using an ELISA, we investigated the levels of IL-5 in the sera and blister fluid of BP patients with active disease and those in prolonged clinical remission treated with intravenous immunoglobulin, and compared it to that in normal controls. Significantly increased levels of IL-5 were detected in the serum and particularly the blister fluid of patients with active disease (P=0.0043) when compared to levels in normal controls. There was no significant difference in IL-5 levels in patients in prolonged clinical remission compared to normal control serum. In an immunoblot assay, using bovine gingival lysate as substrate, we determined the presence of IgG and IgE autoantibodies specific to basement membrane zone proteins in blister fluid and serum of BP patients. IgG autoantibodies to BPAg1 and BPAg2 were detected in both blister fluid and serum of patients, whereas IgE autoantibodies, in much lower titers, were detected to only BPAg1. Elevated levels of blood and tissue eosinophilia were observed in these patients. Based on these data, we present a preliminary hypothesis for the initiation, progression and localization of blister formation in BP.

Autoantibodies↗
PubMed2001

Role of intravenous immunoglobulin in the treatment of bullous pemphigoid: analysis of current data.

BACKGROUND: Conventional therapy of severe bullous pemphigoid (BP) relies on the use of high-dose systemic corticosteroids with or without adjuvants, notably immunosuppressive agents. This approach can result in debilitating and potentially fatal side effects, hence the need to explore alternative therapies. Intravenous immunoglobulin (IVIg) therapy is emerging as one possibility. OBJECTIVE: Our purpose was to determine whether any preliminary conclusion can be drawn about the potential for the use of IVIg to treat patients with severe BP. METHODS: A literature search was done to identify reports, in English-language peer-reviewed journals, on the use of IVIg to treat patients with BP. Reports were examined for information on disease duration, severity, therapies used before and after IVIg, dose and frequency of IVIg administration, and its immediate and long-term effects. RESULTS: Data on treatment of 17 patients indicated that 12 patients (70%) experienced a beneficial clinical response to IVIg. In 5 patients (30%) no clinical benefit was observed. A minimum dose of 2 g/kg per cycle at monthly intervals for 3 months has been the most common approach. However, this should not be perceived as a "standard dose" at the present time. In some patients the use of IVIg appears to permit a systemic corticosteroid-sparing effect. Longer use has achieved sustained clinical remission in some patients. Lack of response was observed in patients who received low-dose IVIg or who received a single infusion only. Minimal side effects in the form of headaches and nausea and vomiting were observed in some patients. CONCLUSION: The present experience consists only of open uncontrolled trials in a few patients and does not allow for definitive conclusions. However, at this time IVIg appears to be a promising agent for the treatment of BP, especially for patients who do not respond to conventional therapy. Adequate doses for longer periods may be required to induce and maintain sustained clinical remissions. Large-scale controlled studies with defined entry criteria, objectives, end points, and long-term follow-up are necessary to determine the specific role of IVIg in the overall management of BP.

Aged↗
PubMed2001

Emerging treatment for epidermolysis bullosa acquisita.

Epidermolysis bullosa acquisita (EBA) is a rare, chronic, subepidermal, mucocutaneous blistering disease characterized by skin fragility and spontaneous as well as trauma-induced blisters that heal with scar formation and milia. Treatment is often frustrating because conventional therapy with corticosteroids and immunosuppressive agents frequently does not result in significant clinical improvement. We review the conventional treatment of EBA and critically analyze the literature on various adjuvants and therapeutic modalities that have recently been used. These include cyclosporine, colchicine, plasmapheresis, extracorporeal photochemotherapy, and intravenous gammaglobulins. Although the data are preliminary, they suggest that intravenous immunoglobulins may be a promising treatment modality for resistant, nonresponsive, or refractory EBA. The use of intravenous immunoglobulins results in significant improvement of skin and mucosal lesions, and it is quite safe, with minimal side effects.

Colchicine↗
PubMed2000

Bovine gingival lysate: a novel substrate for rapid diagnosis of autoimmune vesiculo-bullous diseases. A preliminary observation.

Immunoblot assays have been developed to characterize the autoantigens and to detect autoantibodies in muco-cutaneous autoimmune vesiculo-bullous diseases using different substrates. However the results have been inconsistent, because availability and standardization of different substrates has been a major problem. The aim of this study was to develop an immunoblot assay using bovine gingival lysate as substrate because it is easily and readily available as well as inexpensive. Sera from patients with different vesiculo-bullous diseases were studied. These included 25 patients with pemphigus vulgaris (PV), 8 with paraneoplastic pemphigus (PNP), 12 with pemphigus foliaceus (PF), 25 with bullous pemphigoid (BP), and 22 with cicatricial pemphigoid (CP). Serum samples from 40 normal human volunteers were also studied. The autoantibody titers were determined based on the binding pattern of each disease and compared to those obtained by routine indirect immunofluorescence (IIF). Our observations suggest that the titers from immunoblot assays were significantly higher than titers obtained by IIF (P<0.0001). When the autoantibody titers were compared using bovine gingival lysate and human epidermal lysate as substrate, statistically significant differences were not observed. The use of bovine gingival lysate as a substrate will facilitate the rapid and early serological diagnosis of patients with vesiculobullous diseases. It may also be of benefit to laboratory investigators studying these autoantibodies.

Animals↗
PubMed2000

Nail involvement in pemphigus vulgaris.

Nail involvement in pemphigus vulgaris is relatively rare. We describe a case of severe pemphigus involving both the skin and oral mucosa in which an acute exacerbation was preceded by the onset of nail involvement of all 4 extremities. Nail involvement occurred in the form of hemorrhagic paronychia of multiple digits. Oral, cutaneous, and nail manifestations of the disease were all well controlled by systemic therapy. A review of the literature on nail involvement in pemphigus reveals that this involvement may be manifested in multiple ways, with chronic paronychia and onychomadesis being the most common. Involvement of the nail occurs most frequently either as part of the initial presentation, or just before or concurrent with a flare of pre-existing disease. Nail involvement, when it occurs, is usually present when the disease is severe. Topical therapy is insufficient, and systemic therapy is warranted. In the majority of cases, nail recovery is complete, with no residual damage.

Foot↗
PubMed2000

Analysis of current data on the use of intravenous immunoglobulins in management of pemphigus vulgaris.

BACKGROUND: Systemic corticosteroids, with or without the addition of immunosuppressive adjuvant agents, are frequently used in treating patients with pemphigus vulgaris (PV). The severe, catastrophic, and potentially fatal side effects of these agents highlight the need for the development of safe alternatives for PV therapy. Intravenous immunoglobulin (IVIG) therapy has recently been reported to be effective in the treatment of PV. OBJECTIVE: Our purpose was to do a retrospective analysis of the available literature on the use of IVIG in the treatment of PV. We also wished to determine whether the cumulative evidence permits making preliminary conclusions regarding the potential role of IVIG in the overall management of PV. METHODS: A review of the English-language, peer-reviewed literature was conducted for reports on IVIG use in treatment of PV. The available information on 21 patients was used to assess different dimensions of clinical efficacy. RESULTS: A minimum dose of 2 g/kg per cycle given at regular monthly intervals for a minimum of 3 cycles seems be effective in inducing a rapid clinical remission in patients with severe, recalcitrant PV. However, this should not be perceived as a "standard" dose. Tapering and eventual discontinuation of other agents were possible in many patients. Long-term follow-up was not provided to examine the influence of IVIG on the clinical course of disease, its efficacy as monotherapy, and the benefit of using it as maintenance therapy to keep the patient in prolonged clinical remission. In 4 of the 21 patients (19%), use of IVIG was of no clinical benefit. This failure of efficacy was primarily due to inadequate use. IVIG demonstrated beneficial effect in 17 of 21 patients (81%). CONCLUSION: IVIG may be a safe and effective agent in the management of severe, recalcitrant PV. Multicenter controlled studies, using different dose regimens, with lengthy follow-up periods are necessary to clearly define the emerging beneficial role of IVIG.

Adult↗
PubMed2000

Pemphigoid gestationis: a review.

Pemphigoid gestationis is a rare autoimmune vesiculobullous skin disease closely related to the pemphigoid group of blistering disorders. It is unique in that it is most frequently associated with pregnancy. Diagnosis is made on the basis of the presence of a subepidermal vesicle on routine histologic examination and of linear deposition of the third component of complement along the basement membrane zone of perilesional skin. Abnormal expression of major histocompatibility complex class II molecules in the placenta may trigger the initiating immunologic event in this organ, which is followed by cross-reactivity with an antigen present in the skin and the characteristic cutaneous signs of the disease. The immunogenetics of this disorder are interesting. A role for the involvement of major histocompatibility complex class II antigens seems likely because of the association with human leukocyte antigens DR3 and DR4. Major histocompatibility complex class III associations have also been observed, as has a high frequency of anti-human leukocyte antigen antibodies. We review the current understanding of the molecular biologic and immunogenetic properties of this disease and discuss treatment and potential impact on the fetus.

Diagnosis, Differential↗