Category Archives: Medical

Herpes virus infections are well known infectious complications of pemphigus and bullous pemphigoid. We describe pathologic findings utilizing autopsy tissue from several organs from a patient affected by a new variant of endemic pemphigus in El Bagre, Colombia, South America.

We describe a patient by a new variant of endemic pemphigus foliaceus from El Bagre that was receiving high-dosage immunosuppressants when hospitalized and died suddenly following contact with a second patient affected by chicken pox.

We performed studies utilizing hematoxylin and eosin, immunohistochemistry, and direct immunofluorescence techniques on tissues from several organs.

We detected the presence of varicella zoster virus, as well as strong positivity for α-1 antitrypsin in the heart, kidneys, spleen, liver, skin, brain, lungs, pancreas, small and large intestines, and skeletal muscle. In regard to structural damage in the kidney and heart, we believe the observed damage is associated with the presence of autoantibodies to these organs, since both of them are rich in plakins and El Bagre-EPF patients present significant antibodies to plakin molecules.

In patients with endemic pemphigus foliaceus, we recommend complete isolation of the patient when receiving high dosages of systemic immunosuppressive agents. We further suggest the clinical possibility of a synergistic, fatal interaction between active pemphigus foliaceus, varicella zoster virus, herpes simplex virus, immunosuppressive agents, and a systemic activation of α-1 antitrypsin. Thus, we suggest adequate bed spacing, barrier nursing, and preventative testing for α-1 antitrypsin activation are warranted in these patients to address these complications.

 

Source: http://onlinelibrary.wiley.com/doi/10.1111/j.1365-4632.2011.05296.x/abstract

Background:  Pemphigus vulgaris (PV) is an autoimmune blistering skin disorder characterized by the presence of suprabasal acantholysis and autoantibodies against desmoglein 3. There are two different clinical forms: mucocutaneous (MCPV) or mucosal (MPV). However, it is not clear how PV lesions in oral, ear, nose and throat (OENT) areas produced by the very dynamic of the anatomical structures involved in the functions of the aerodigestive tract.

Objectives:  To investigate the pattern of OENT manifestations in PV, and their relationship with physiological traumatic mechanisms in stratified squamous epithelium structures.

Patients:  A prospective analysis of 40 patients diagnosed with MCPV (22 patients) or MPV (18 patients) was carried out in the University Clinic of Navarra. OENT manifestations were evaluated in all patients endoscopically. OENT involvement was divided into anatomical areas.

Results:  The most frequent symptom was pain, mainly on oral mucosa (87,5%). Buccal mucosa (90%), posterior wall of pharynx (67.5%), upper edge of epiglottis (85%) and nasal vestibule (70%) were the areas most frequently affected in the OENT mucosa. These localizations were related to physiological traumatic mechanisms in polystratified squamous epithelium structures.

Conclusions:  OENT endoscopy should be included in the examination of all PV patients. To know the most frequent localizations of active lesions on OENT mucosa in PV will help us to interpreter more efficiently the findings from OENT endoscopy. Also, information related to traumatic physiological mechanisms on OENT areas must be offered to patients in order to avoid the appearance of new active PV lesions.

 

Source: Study of Oral, Ear, Nose, and Throat Involvement…

Bullous pemphigoid (BP) is an autoimmune blistering skin disease. Auto-antibodies to BP180 and BP230 can be detected by indirect immunofluorescence (IIF) on different substrates (oesophagus, salt-split-skin, BP180-antigen dots, BP230-transfected cells) and ELISA. Here, we compared test characteristics of these test systems. We analyzed sera from BP patients (n=60) in whom the clinical diagnosis had been confirmed histopathologically. The control cohort comprised sera from patients with other autoimmune-associated (n=22) or inflammatory (n=35) skin diseases. All samples were tested by IIF (EUROIMMUN™ Dermatology Mosaic) and ELISA (EUROIMMUN and MBL). Anti-BP180 is best detected with BP180-antigen dots by IIF (sensitivity: 88%; specificity: 97%). As compared to IIF, the differences with both BP180 ELISA techniques are small though. Likelihood ratios (LRs) for positive and negative test results are >10 and between 0.1 and 0.2, respectively, for all test systems. Detection of anti-BP230 is highly variable (sensitivity range 38-60%; specificity range 83-98%). Only the IIF test reveals a LR for positive test results >10. Since the LRs for a negative test are all ~0.5, negative test results for anti-BP230 antibodies do not help to exclude BP. In conclusion, the multi-parameter IIF test reveals a good diagnostic performance in BP. Since this test simultaneously allows for the detection of anti-Dsg1 and anti-Dsg3 antibodies, involved in pemphigus foliaceus and vulgaris, a single test-incubation may be sufficient to differentiate between the most frequent autoimmune blistering diseases.

Copyright © 2012 Elsevier B.V. All rights reserved.

 

Source: http://www.ncbi.nlm.nih.gov/pubmed/22580378?dopt=Abstract

By Rebecca Berman, Janet Segall and Jean-Claude Bystryn, M.D. from The National Pemphigus Foundation and The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, NY. February 17, 1999

As many of you know, we recently conducted a survey of persons with pemphigus to find out 1) which treatments were most commonly used, 2) which appeared to be the most effective, and 3) which were most often associated with side effects. The survey was conducted by means of a questionnaire enclosed in the Fall 1998 issue of the National Pemphigus Foundation newsletter, the Quarterly. The questionnaire was also sent to all individuals who responded to a notice on the NPF website.A total of 110 responses were received. This number is impressive, taking into account the rarity of pemphigus. We thank all participants for their collaboration.

Intravenous immune globulin (IVIG) preparations are efficacious and safe products in use world-wide. Although rare, side-effects of IVIG may be serious, even life-threatening, and clinicians should be aware of their potential occurrence.

The clinical benefit of immune globulin prophylaxis in patients
with primary antibody deficiency syndromes has been clearly
established. In the past, replacement therapy was provided
through intramuscular injections. In the early 1980s, highly
purified monomeric suspensions of IgG for intravenous use
became available and more than 10 commercial preparations
of intravenous immune globulin (IVIG) are now at the disposal
of the clinician. The indications for administration of
IVIG have been enlarged to include transitory primary antibody
deficiencies (such as low birth-weight premature babies),
secondary hypogammaglobulinaemic states [as in chronic
lymphatic leukaemia (CLL) or multiple myeloma], and conditions
with increased susceptibility to infections (such as bone
marrow transplant.or the post-surgery period). In addition to
its efficacy as replacement therapy, IVIG now has wellestablished
therapeutic applications in some haematological
and autoimmune diseases: IVIG preparations are used successfully
in immune thrombocytopenic purpura (ITP), in Kawasaki
disease, and for some desperate diseases for which there is no
other efficient treatment [reviewed in refs 1 and 2]. The mechanisms
of action of IVIG in these conditions, although not yet
fully determined, include a reticulo-endothelial blockade, an
immunomodulatory effect (by supplying anti-idiotype antibodies),
and an anti-inflammatory action.
This growing usage has increased the need for high quality
immune globulin products and, indeed, high-dose IVIG can be
administered with only mild, self-limited side-effects. This
paper reviews the most frequent adverse reactions reported
with IVIG therapy from the time of its introduction into the
clinic. Possible underlying causes of these reactions and their
current management are described briefly.

To read the rest of this article, click on the link: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1550378/pdf/clinexpimmunol00030-0077.pdf

Background

Pemphigus vulgaris (PV, OMIM 169610) is a severe blistering disorder of the skin and mucous membranes, caused by the production of autoantibodies directed against the epithelial adhesive protein desmoglein 3. Although an association between PV and HLA class II alleles has been established, the genetic factors predisposing to the disease remain poorly understood, the rarity of PV hampering the recruitment of substantial patient cohorts.

Objectives

To investigate DSG3 as a candidate PV susceptibility gene.

Methods

We examined five DSG3 single nucleotide polymorphisms (rs8085532, rs3911655, rs3848485, rs3794925 and rs1466379) in two case–control datasets respectively originating from the U.K. (62 PV patients, 154 controls) and northern India (28 patients, 98 controls). Results In the U.K. sample, we observed a significant association between PV and the DSG3*TCCTC haplotype (Fisher’s exact test P ¼ 0Æ002). A related haplotype (DSG3*TCCCC) was associated with PV in the Indian dataset (P ¼ 0Æ002). We also found that all British and Indian patients bearing DSG3 risk haplotypes carried at least one copy of a PV-associated HLA allele.

Conclusions

These results suggest that genetic variation of DSG3 may be an additive risk factor predisposing to PV and warrant further investigations of this gene.

Published in the British Journal of Dermatology 2006

PV Study in BJOD