Tag Archives: bullous pemphigoid

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The U.S. Food and Drug Administration (FDA) has granted Fast Track Designation to bertilimumab for the treatment of bullous pemphigoid. The FDA’s Fast Track program is designed to facilitate the development and expedite the review of drugs to treat serious conditions that are unmet medical needs.

Pemphigus and pemphigoid (P/P) patients may agree that in the early months of their condition it is a very painful and difficult time. However, the following suggestions may be helpful like learning how to care for our skin in terms of taking and applying topical medications, cleansers, moisturizers, dressings, and staying comfortable.

While you are seeing a qualified dermatologist who is treating you for your Pemphigus Vulgaris, Bullous Pemphigoid, Pemphigus Foliaceus, Mucous Membrane Pemphigoid, etc. you might also be seeing your own dentist, OB/GYN, internist, ophthalmologist or ear/nose/throat specialist.

Please be sure that all of your doctors are aware of your condition and that they have access to your dermatologist.  It is important that they know the medications and dosage that you are taking for each medication.

All of your doctors need to be able to communicate with one another if necessary.  Being left in the dark will leave you at a disadvantage.  Also, if you are going to be scheduled for any major dental work, advise your dermatologist.  Depending on the procedure, your medications may be adjusted for a few days prior and a few days following to prevent any flare-ups.

Remember when you need us we are in your corner!

The relationship between bullous pemphigoid (BP) and neurological disease has been the subject of numerous recent studies and BP antigens and their isoforms have been identified in the central nervous system (CNS). Whilst epidemiological data support this association, little is known about the pathomechanism behind this link and the immunological characteristics of patients with BP and neurological disease, other than multiple sclerosis (MS), has not been studied. We aimed to compare the cutaneous immune response in BP patients with and without neurological disease, to investigate whether or not there is a distinctive immunopathological profile in patients with concomitant BP and neurological disease. Seventy-two patients with BP were included and divided into two groups; those with neurological disease (BP+N, n = 43) and those without (BP−N, n = 29).

Patients in BP+N group had a confirmed neurological disease by a hospital physician, neurologist or psychiatrist with positive neurological imaging where appropriate, or a Karnofsky score of 50 or less due to mental impairment. All sera were analysed with indirect immunofluorescence (IIF) using serial dilutions up to 1:120000, immunoblotting (IB) and Enzyme-linked immunosorbent assay (ELISA) for BP180 and BP230. Median antibody titres by IIF were 1:1600 vs. 1:800 for BP−N and BP+N, respectively, although the difference did not reach statistic significance (P = 0.93, Mann–Whitney U-test).

ELISA values for both BP180 and BP230 did not differ significantly between the two groups. Similarly, autoantibodies to specific antigens as identified by ELISA and IB were not related to the presence of neurological disease. The results of this study indicate that patients with BP and neurological disease exhibit an immune response to both BP180 and BP230, thus the link between the CNS and the skin is not dependent on a specific antigen, but possibly both antigens or their isoforms may be exposed following a neurological insult, and play a role in generation of an immune response. 

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Bullous pemphigoid is an autoimmune blistering skin disease characterized by the presence of circulating autoantibodies which recognize specific proteins of the epidermis and dermoepidermal junction. Diagnosis is based on clinical criteria and laboratory investigations, notably histology, direct and indirect immunofluorescence, and ELISA. This study describes a new immunofluorescence assay for parallel determination of anti-BP180 and anti-BP230 based on recombinant antigenic substrates. The aim of the study was to detect BP180 and BP230 autoantibodies by BIOCHIP technology using both a specially designed recombinant BP180-NC16A protein and cells expressing the BP230-gc antigen fragment. 18 patients with bullous pemphigoid were included in the study. Autoantibodies to BP180 were detected by the BIOCHIP technique in 83.33% of patients with clinical, serological, and immunohistological confirmed bullous pemphigoid while autoantibodies against BP230-gC were detected only in 39% of patients. The detection of anti-BP180-NC16A and anti-BP230-gC by a new biochip-based immunoassay is a suitable alternative to indirect immunofluorescence and ELISA. This method has the advantage of easily discriminating the different autoantibody specificities. The BIOCHIP method is faster, cheaper, and easy to use when compared with the ELISA approach. For this reason, the new method could be used as an initial screening test to identify patients with bullous pemphigoid, and doubtful results could then be confirmed by ELISA.

Full article (free) found here: http://www.hindawi.com/isrn/dermatology/2012/237802/

To evaluate the significance of the association of malignancy with autoimmune blistering diseases, we studied the incidence of internal malignancies in pemphigus and bullous pemphigoid based upon 496 cases of pemphigus and 1113 cases of bullous pemphigoid in Japan. Results showed that (1) an association between internal malignancies and pemphigus was observed in 25 out of 496 cases (5.0%), while that with bullous pemphigoid was seen in 64 out of 1113 cases (5.8%). Such association ratios were significantly higher than that of the controls aged over 70 years old (0.61%); (2) The average ages of pemphigus/bullous pemphigoid with malignancy were 64.7 and 69.2 years, respectively. The association ratio of malignancy with pemphigus increased by age, while that with pemphigoid was not correlated with aging; (3) Lung cancer was most common in pemphigus and gastric cancer in bullous pemphigoid; (4) There were no significant differences in the titers of circulating antibody, the presence or extent of mucous involvement or annular erythema between bullous pemphigoid patients with malignancy and without malignancy. Our results indicated that detailed examination for internal malignancy is essential for those patients with pemphigus or bullous pemphigoid.

Abstract from: http://www.ncbi.nlm.nih.gov/pubmed/7772576

In BP lesional skin, immunohistochemistry and confocal microscopy were performed for CD4+, CD25+, forkhead/winged helix transcription factor (FOXP3)+, transforming growth factor (TGF)-β+ and interleukin (IL)-10+ cells. In addition, the number of CD4+CD25++FOXP3+ Tregs in peripheral blood was assessed by flow cytometry, and the levels of TGF-β and IL-10 were determined in serum samples by enzyme-linked immunosorbent assay before and after steroid therapy. Controls included patients with psoriasis, atopic dermatitis (AD) and healthy donors.

The frequency of FOXP3+ cells was significantly reduced in skin lesions from patients with BP (P < 0.001) compared with psoriasis and AD. Moreover, the number of IL-10+ cells was lower in BP than in psoriasis (P < 0.001) and AD (P = 0.002), while no differences were observed in the number of TGF-β+ cells. CD4+CD25++FOXP3+ Treg in the peripheral blood of patients with BP was significantly reduced compared with healthy controls (P < 0.001), and augmented significantly after steroid therapy (P = 0.001). Finally, TGF-β and IL-10 serum levels were similar in patients with BP compared with healthy controls. However, after therapy, BP patients showed significantly higher IL-10 serum levels than before therapy (P = 0.01).

Full article available at: http://onlinelibrary.wiley.com/doi/10.1111/jdv.12091/abstract;jsessionid=C37D521517222D9766F5D0D339765626.d04t01?deniedAccessCustomisedMessage=&userIsAuthenticated=false

Antiga, E., Quaglino, P., Volpi, W., Pierini, I., Del Bianco, E., Bianchi, B., Novelli, M., Savoia, P., Bernengo, M.G., Fabbri, P. and Caproni, M. (2013), Regulatory T cells in skin lesions and blood of patients with bullous pemphigoid. Journal of the European Academy of Dermatology and Venereology. doi: 10.1111/jdv.12091
Background  Bullous skin diseases are known to be associated with significant morbidity and mortality. There have been no studies on mortality from severe bullous skin diseases in Canada.

Methods  We used mortality data from the Statistics Canada website from 2000 to 2007 for three major bullous skin diseases: bullous pemphigoid; pemphigus; and toxic epidermal necrolysis (TEN). Crude and age-standardized mortality rates were calculated and compared with the corresponding US mortality rates. Linear regression was used to assess time trend and effect of gender and age on mortality rates.

Results  During the period of eight years, there were 115 deaths attributed to pemphigoid, 84 to pemphigus, and 44 to TEN. The crude annual mortality rate was the highest for pemphigoid (0.045 per 100,000), followed by pemphigus (0.033), and TEN (0.017). None of these conditions demonstrated significant time trends in mortality rates over the eight-year period, although a trend towards decreasing pemphigus mortality was observed (P = 0.07). No gender difference in mortality was observed, but advanced age was associated with mortality in all three conditions.

Conclusion  Among bullous skin diseases, pemphigoid is the leading cause of mortality in Canada. This is in contrast to the USA, where TEN is the leading cause of mortality from bullous skin diseases. It is not clear whether differences in healthcare systems explain these findings.

Full article available at: http://onlinelibrary.wiley.com/doi/10.1111/j.1365-4632.2011.05227.x/abstract;jsessionid=FAE06EFE4AF802D50261B2992F71D91D.d02t01?systemMessage=Wiley+Online+Library+will+be+disrupted+on+27+October+from+10%3A00-12%3A00+BST+%2805%3A00-07%3A00+EDT%29+for+essential+maintenance

MADAM, Autoantibodies in pemphigus target preferentially desmoglein 1 (Dsg1) and Dsg3, and rarely desmocollins 1-3 (Dsc1-3). Pemphigus herpetiformis (PH) is one of pemphigus subtypes and characterized by pruritic annular erythemas with vesicles in the periphery, rarity of mucosal involvement and histopathological change of eosinophilic spongiosis. Recently, IgG anti-Dsc3 autoantibodies were suggested to cause skin lesion in a case of pemphigus vulgaris. In this study, we report the first case of concurrent bullous pemphigoid (BP) and PH with IgG antibodies to both Dsgs and Dscs.

from: http://onlinelibrary.wiley.com/doi/10.1111/bjd.12019/abstract