Tag Archives: pemphigus foliaceus

Principia Biopharma Inc. (Nasdaq: PRNB), a clinical-stage biopharmaceutical company dedicated to bringing transformative oral therapies to patients with significant unmet medical needs in immunology and oncology, today announced positive top-line data from the completed open-label Phase 2 trial of PRN1008 in patients with pemphigus (including both pemphigus vulgaris (PV) and pemphigus foliaceus (PF)) and the initiation of a Phase 3 trial of PRN1008 in pemphigus.

Syntimmune recently announced positive preliminary results from its phase 1b proof-of-concept trial of SYNT001 in pemphigus vulgaris and foliaceus patients. It’s exciting for the IPPF to share good news related to research and treatments. The full press release from Syntimmune can be found here. The following is an excerpt:

 

Syntimmune, Inc., a clinical-stage biotechnology company developing antibody therapeutics targeting FcRn, today announced positive preliminary results from its Phase 1b proof-of-concept trial of SYNT001 in patients with pemphigus vulgaris and pemphigus foliaceus. The data showed clinically meaningful benefit of SYNT001, with a favorable safety and tolerability profile similar to that observed in the Phase 1a study.

“There remains a clear unmet need for a safe and fast-acting treatment for patients with pemphigus, who face serious symptoms and complications associated with their disease,” said Donna Culton, M.D., Ph.D., an assistant professor at the University of North Carolina School of Medicine. Culton presented preliminary results of the Phase 1b study at the International Investigative Dermatology conference being held on May 16-19, 2018 in Orlando, FL. “These preliminary data demonstrate safety as well as a rapid reduction in PDAI scores and lowering of IgG levels with treatment of SYNT001, which support further studies of this drug as a potential new therapeutic option,” Culton said.

Read Syntimmune’s press release, including additional information, here. 

Photo of Rudy Soto with his family.

My name is Rudy Soto. I am from the great state of Texas and have lived there all of my life. I am married to a wonderful woman, Jennifer, who is my greatest supporter. We have four awesome children—two girls and two boys who range in age from 5 to 23. My motto is Can’t Grind Me Down, which I took on after I achieved remission in November of 2016.

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!

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, 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

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.

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 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

Context.-Pemphigus is a group of autoimmune vesiculobullous diseases characterized by immunoglobulin G (IgG) antibodies directed against desmosomal adhesion proteins, with IgG4 being the predominant subclass in active diseases. Direct immunofluorescence for IgG performed on fresh-frozen tissue plays a crucial role in diagnosing pemphigus. However, the diagnosis might be hindered when frozen tissue is not available. Objective.-To evaluate the usefulness of immunohistochemistry for IgG4 performed on paraffin sections as a diagnostic test for pemphigus. Design.-Eighteen immunofluorescence-proven pemphigus cases (12 pemphigus vulgaris, 6 pemphigus foliaceus) were studied. Four normal skin specimens and 32 nonpemphigus vesiculobullous disease specimens served as controls. Paraffin sections of all cases were examined immunohistochemically for IgG4 expression. Positivity was defined as distinct, condensed, continuous immunoreactivity localized to the intercellular junctions of keratinocytes. Results.-The immunostains were independently evaluated in a masked manner by 3 pathologists, with a 100% interobserver agreement. Nine of 12 pemphigus vulgaris cases (sensitivity 75.0%), and 4 of 6 pemphigus foliaceus cases (sensitivity 66.7%), were positive for IgG4 immunostain. The overall sensitivity was 72.2%. One control specimen (bullous pemphigoid) showed IgG4 positivity (specificity 97.2%). In specimens demonstrating acantholysis, 8 of 10 pemphigus vulgaris cases (sensitivity 80.0%) and 4 of 4 pemphigus foliaceus cases (sensitivity 100.0%) were positive for IgG4. The overall sensitivity for specimens with acantholytic lesions was 85.7%. Conclusion.-Immunohistochemistry for IgG4 provides a reasonably sensitive and highly specific test for diagnosing pemphigus, especially when frozen tissue is not available, and active acantholytic lesions are examined.

Full article available at: http://www.ncbi.nlm.nih.gov/pubmed/23106586?dopt=Abstract

A 14-year-old male presented with seven years history of recurrent episodes of fluid filled, itchy and eroded lesions over the body not responding to oral corticosteroids and azathioprine. Dermatological examination revealed crusted plaques and erosions in a seborrheic distribution. Histopathology of skin lesions and direct immunofluorescence were characteristic of pemphigus foliaceus. He was treated with dexamethasone pulse therapy with inadequate response. However, relapsing skin lesions revealed a circinate arrangement with a predilection to trunk and flexures. In view of clinical features suggestive of IgA pemphigus, he was started on dapsone, to which he responded dramatically in four weeks. However, repeat biopsy continued to reveal features of pemphigus foliaceus and ELISA for anti- desmoglein 1 antibodies was positive.

Background.  Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are autoimmune vesicobullous disorders with IgG autoantibodies directed against desmoglein (Dsg)1 and 3, which lead to intraepidermal acantholysis.

Aim.  To characterize the clinical and immunological profile of patients with PF or PV with umbilical involvement.

Methods.  In total, 10 patients (7 women, 3 men; age range 24–70 years, disease duration 3–16 years) diagnosed with either PV (n = 5) or mucocutaneous PF (n = 5) were assessed according to their clinical features, histopathology and immunological findings .

Results.  Erythema, erosions, crusts and vegetating skin lesions were the main clinical features of the umbilical region. DIF of the umbilical region gave positive results for intercellular epidermal IgG and C3 deposits in eight patients and for IgG alone in the other two. Indirect immunofluorescence with IgG conjugate showing the typical pemphigus pattern was positive in all 10 patients, with titres varying from 1 : 160 to 1 : 2560. ELISA with recombinant Dsg1 gave scores of 24–266 in PF and 0–270 in PV. Reactivity to recombinant Dsg3 was positive in all five patients with PV (ELISA 22–98) and was negative in all PF sera.

Conclusions.  All 10 patients with pemphigus with umbilical presentation had the clinical and immunopathological features of either PF or PV. This peculiar presentation, not yet completely elucidated, has rarely been reported in the literature. A possible explanation for this unique presentation may be the presence of either novel epitopes or an association with embryonic or scar tissue located in the umbilical-cord region.

Full article available at: http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2230.2012.04468.x/abstract