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1-s2.0-S0929664612005104-gr2Lobar torsion is a rare complication after lung transplantation. Here we report a case of right middle lobe (RML) torsion after bilateral sequential lung transplantation (BLTx). This 30-year-old lady underwent BLTx for bronchiolitis obliterans due to paraneoplastic pemphigus. The right lower lobe of the donor lung was resected due to inflammatory change during procurement. The postoperative chest X-ray showed persisting RML infiltrates. Fever and leukocytosis were noted 1 week later. RML lobectomy was performed after the reconstructed chest computed tomography confirmed the diagnosis of RML torsion. Adult respiratory distress syndrome with unstable vital signs, refractory hypoxemia and respiratory acidosis occurred thereafter. After venoarterial extracorporeal membrane oxygenation support, the patient recovered slowly and was discharged 5 months after BLTx.

Full article can be purchased here: http://www.sciencedirect.com/science/article/pii/S0929664612005104

Pemphigus foliaceus (PF) is the most common autoimmune skin disease of dogs and other animal species. Although PF can spontaneously affect dogs of any breed, it appears more prevalent in Akita Inus and chow chows in the United States. The primary lesions are large pustules which rupture easily and progress rapidly to erosions and crusts. Lesion distribution most often involves the face, nasal planum, and ears. One third of affected dogs have paw pad lesions. Skin lesions of PF can remain localized or involve the entire body. The diagnosis of PF in dogs is based on historical information, clinical signs,

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and the demonstration of acantholytic keratinocytes in vesicles or pustules. (Source: Advances in Small Animal Medicine and Surgery)

Article can be purchased from : http://www.advancesinsmallanimal.com/article/PIIS1041782612000230/abstract?rss=yes

BACKGROUND: Pemphigus is an autoimmune blistering disease. According to a report, in areas of endemic pemphigus foliaceus (EPF) in Peru there are cases of pemphigus vulgaris with epidemiologic, clinical and histopathologic characteristics similar to those of “endemic pemphigus vulgaris” (EPV) in Brazil.
OBJECTIVES: To determine the clinical and epidemiologic characteristics of endemic pemphigus and the risk factors of patients for developing complications during treatment.
METHODS: A study was carried out from July 2003 to March 2008. The study population was 60 patients with EPF and 7 patients with EPV evaluated in hospitals and clinics in the Peruvian Amazon and Lima. A multivariate analysis was carried out using binary logistic regression.
RESULTS: The average age of EPF patients was 31.4 years; 55% were men; 60% presented the generalized clinical variant. Non-compliance with the treatment was seen in 57.1% of the patients. Thirty-five percent presented complications (e.g. pyodermitis and pyelonephritis) during treatment. The risk factors for developing complications during treatment were non-compliance with the treatment and having the generalized clinical form. In the EPV group, the average age was 21.7 years; 71.4% were men. All patients presented with the mucocutaneous clinical variant and the initial presentation consisted of oral mucosa lesions; 71.4% presented complications during treatment, pyodermitis being the most frequent.
CONCLUSIONS: Non-compliance with the treatment and the generalized clinical form are risk factors for the development of complications during treatment of patients with EPF. Peru indeed has EPV cases with epidemiologic characteristics similar to EPF. Living in a rural area may represent a risk factor for the development of complications during treatment of patients with EPV.

Full article can be viewed here: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962012000600003&lng=en&nrm=iso&tlng=en

Paraneoplastic pemphigus (PNP), a clinically and immunopathologically distinct mucocutaneous blistering dermatosis, is a severe form of autoimmune multiorgan syndrome generally associated with poor therapeutic outcome and high mortality. This IgG-mediated disease is initiated by an obvious or occult lymphoproliferative disorder in most cases. Clinically severe mucositis, and polymorphic blistering skin eruptions, and histologically acantholysis, keratinocyte necrosis and interface dermatitis are its hallmark features. A 58-year-old female presented with recurrent, severe, recalcitrant stomatitis and widespread erosions/blistering lesions of one-year duration. Treatment with repeated courses of systemic corticosteroids at a peripheral center would provide temporary relief. She also had fever, productive cough, odynophagia and poor oral intake, herpes zoster ophthalmicus, pain in the abdomen, and watery diarrhea. An array of investigations revealed chronic lymphocytic leukemia (CLL), mediastinal and para-aortic lymphadenopathy, bronchiolitis obliterans, and vertebral osteoporosis/fractures. With the diagnosis of CLL-associated PNP she was managed with dexamethasone-cyclophosphamide pulse (DCP) therapy for 3 cycles initially, followed by COP regimen (cyclophosphamide, vincristine, and prednisolone) for 5 cycles. Remission is being maintained with chlorambucil and prednisolone pulse therapy once in 3 weeks with complete resolution of skin lesions and adequate control of CLL.

Full article can be viewed here: http://www.hindawi.com/crim/dm/2012/207126/

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/

There are a limited number of reports indicating the role of human leukocyte antigen (HLA) class I alleles in pemphigus vulgaris. This study was designed to highlight the association of HLA class I alleles with pemphigus vulgaris in Iran. Fifty patients with pemphigus vulgaris, diagnosed based on clinical, histological and direct immunofluorescence findings were enrolled into this study. The control group consisted of 50 healthy, age- and sex-matched individuals. HLA typing of class I (A, B and C alleles) was carried out using polymerase chain reaction based on the sequence-specific primer method. This study showed the higher frequency of HLA-B*44:02 (= 0.007), -C*04:01 (< 0.001), -C*15:02 (< 0.001) and -C*16:01 (= 0.027) in the patient group, compared to the controls, while the frequency of HLA-C*06:02 (< 0.001) and -C*18:01 (= 0.008) in the patients with pemphigus vulgaris was significantly lower than the controls. Regarding the linkage disequilibrium between HLA class I alleles, the HLA-A*03:01, -B*51:01, -C*16:02 haplotype (4% vs 0%,= 0.04) is suggested to be a predisposing factor, whereas HLA-A*26:01, -B*38, -C*12:03 haplotype (0% vs 6%, = 0.01) is suggested to be a protective factor. In conclusion, it is suggested that HLA-B*44:02, -C*04:01, -C*15:02 alleles and HLA-A*03:01, -B*51:01, -C*16:02 haplotype are susceptibility factors for development of pemphigus vulgaris in the Iranian population, while HLA-C*06:02, -C*18:01 alleles and HLA-A*26:01, -B*38, -C*12:03 haplotype may be considered as protective alleles.

Full article available here: http://onlinelibrary.wiley.com/doi/10.1111/1346-8138.12071/abstract;jsessionid=B90D811159F2CE1C4C357306A37A9D15.d04t04

american-quarter-horsePemphigus foliaceus (pem-fi-gus foli-a-shus) is an auto immune disease that affects humans and dogs and, to a lesser extent, cats and horses.
In horses, it is characterized by primary lesions that often begin on the head and lower extremities; secondary lesions spread to other areas, with an exudate that dries to a crust. There may be extensive edema (swelling) in the legs and abdomen (called “ventral” edema).
Equine pemphigus foliaceus (EPF) is considered rare and signs and symptoms may resemble those of other conditions such as insect bite allergies (crusty lesions), pigeon fever (ventral edema) or other skin conditions.
The primary way to diagnose EPF is by punch biopsy of the skin which is examined by a veterinary pathologist. The pathologist looks for changes consistent with this diagnosis, while also ruling out other causes.
Horses with EPF may also have systemic signs of illness – fever, depression, loss of appetite, lethargy and weight loss. The skin may be painful to touch and swelling can make it difficult to walk or lie down.
Senear-Usher syndrome or pemphigus erythematosus is a pathology that overlaps clinically and serologically with pemphigus foliaceus and lupus erythematosus. Skin biopsies of patients with pemphigus erythematosus reveal acantholysis and deposits of immunoglobulins in desmosomes, and they are positive in the lupus band test. In the present paper, we determined whether the autoantibodies associated with pemphigus erythematosus targeted a single antigen or multiple antigens as a result of the stimulation of independent B cell clones. Our present paper demonstrates that patients with pemphigus erythematosus produce both antiepithelial antibodies specific for desmoglein 1 and 3 and antinuclear antibodies specific for Ro, La, Sm, and double-stranded DNA antigens. After eluting specific anti-epithelial or anti-nuclear antibodies, which were recovered and tested using double-fluorescence assays, a lack of cross-reactivity was demonstrated between desmosomes and nuclear and cytoplasmic lupus antigens. This result suggests that autoantibodies in pemphigus erythematosus are directed against different antigens and that these autoantibodies are produced by independent clones. Given these clinical and serological data, we suggest that pemphigus erythematosus behaves as a multiple autoimmune disease.

Full article can be viewed on: http://www.hindawi.com/journals/ad/2012/296214/

Screenshot_2Pemphigus foliaceus (PF) is an immune-mediated disease that causes pustules and crusted lesions, most commonly on the pinnae, nasal planum, periocular area, chin, feet of affected cats. Acantholytic cells caused by dehydration of intercellular adhesions are often seen on cytology but are not pathognomic for PF. A definitive diagnosis is made based on histopathology showing subcorneal pustules with nondegenerate neutrophils and acantholytic cells. PF is treated with immunosuppressive doses of corticosteroids alone or in combination with other immunosuppresive medications, such as chlorambucil or cyclosporine. Most patients require lifelong treatment with these medications to keep the disease in remission.

Hershey, a 6-year-old, spayed domestic shorthaired cat weighing 3.4 kg, presented with an acute onset of nonpruritic crusted lesions on the head, ears, nail beds, and nasal

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area. She had a 2-day history of lethargy and anorexia. She had no history of medical disease and was up-to-date on vaccinations.

Full article on: http://mobile.vetlearn.com/Media/images/pdf/2010/PV/PV0510_mckay_Derm.pdf

Puppy-Love-puppies-9460996-1600-1200Pemphigus in Dogs

Pemphigus is the general designation for a group of autoimmune skin diseases involving ulceration and crusting of the skin, as well as the formation of fluid-filled sacs and cysts (vesicles), and pus filled lesions (pustules). Some types of pemphigus can also affect the skin tissue of the gums. An autoimmune disease is characterized by the presence of autoantibodies that are produced by the system, but which act against the body’s healthy cells and tissues — just as white blood cells act against infection. In effect, the body is attacking itself. The severity of the disease depends on how deeply the autoantibody deposits into the skin layers. The hallmark sign of pemphigus is a condition called acantholysis, where the skin cells separate and break down because of tissue-bound antibody deposits in the space between cells.

There are four types of pemphigus that affect dogs: pemphigus foliaceus, pemphigus erythematosus, pemphigus vulgaris, and pemphigus vegetans.

In the disease pemphigus foliaceus, the autoantibodies are deposited in the outermost layers of the epidermis, and blisters form on otherwise healthy skin. Pemphigus erythematosus is fairly common, and is a lot like pemphigus foliaceus, but less afflictive. Pemphigus vulgaris, on the other hand, has deeper, and more severe, ulcers because the autoantibody is deposited deep in the skin. Pemphigus vegetans, which affects only dogs, is the rarest form of pemphigus, and seems to be a gentler version of pemphigus vulgaris, with somewhat milder ulcers.

full article can be found here: http://www.petmd.com/dog/conditions/skin/c_dg_pemphigus?page=show#.UQbd3R3WLXA