Authors: Sousa JX, Diaz LA, Eaton DP, Hans-Filho G, Lanzani de Freitas E, Delgado L, Ichimura LM, Cristaldi F, Orlandi R, Kesper N, Umezawa ES, Rivitti EA, Aoki V, The Cooperative Group on Fogo Selvagem Research Abstract Fogo Selvagem (FS) is an autoimmune bullous disease with pathogenic IgG autoantibodies recognizing desmoglein 1 (Dsg1), a desmosomal glycoprotein. In certain settlements of Brazil, a high prevalence of FS (3%) is reported, suggesting environmental factors as triggers of the autoimmune response. Healthy individuals from endemic areas recognize nonpathogenic epitopes of Dsg1, and exposure to hematophagous insects is a risk factor for FS. Fogo selvagem and Chagas disease share some geographic sites, and anti-Dsg1 has been detected in Chagas patients. Indeterminate Cha…
With Pemphigus and Pemphigoid, painful oral lesions frequently occur in the mouth and throat causing difficulty with drinking and eating solid foods. Let’s face it, it can be just painful! Assessing your individual tolerance to foods and adjusting what you are eating will enable you to better understand which foods to eat and which foods to avoid. For many, highly seasoned, acidic, or salty foods are irritating and those foods that are dry, sticky, or abrasive can be difficult to swallow. Extremes in temperatures of liquids or solids such as ice cream or hot chocolate may cause pain for some. In the case of severe mouth sores, use a blender or food processor is accessible.
Here are a few helpful hints to remember:
- Drink liquids through a straw.
- Cook coarse or hard foods, such as vegetables until they are soft and tender.
- Soften or moisten foods by dipping them in gravies or cream sauces.
- Take a swallow of a beverage with solid food.
- Eat small frequent servings rather than a large amount of food at one time.
- Rinse your mouth with water, peroxide, or Biotene during and after eating to help
- Remove food and bacteria and to promote healing.
Having oral lesions can present many challenges including; pain management, oral hygiene, nutritional intake, and your overall health. Speak with your doctor about ways to help relieve the pain and ensure that they are monitoring you blood sugar levels if you are taking systemic steroids. Make sure that you inform your dentist of your condition and require them to use caution when treating you.
If you have difficulty swallowing and find yourself choking on food, see your doctor immediately. Ask to be seen by an ENT so they can help determine the extent of your disease activity. The mouth is often one of the most difficult areas to treat and requires diligence. Changing your behavior and habits can be the biggest “pain” but will eventually pay off. If you need help, encouragement, or suggestions…just “Ask a Coach“!
When you need us, we are in your corner!
Marc Yale – Peer Health Coach
Background Pemphigus foliaceus (PF) is a chronic cutaneous autoimmune blistering disease that is characterized by superficial blistering of the skin, and according to the current perspective is caused by autoantibodies directed against desmoglein 1 (Dsg1).
Objectives To examine early acantholysis in skin of PF patients at an ultrastructural level.
Methods Two Nikolsky negative (N-), five Nikolsky positive (N+) and two lesional skin biopsies of immunoserological defined PF patients were studied by light and electron microscopy.
Results We found no abnormalities in N- PF skin, whereas all N+ skin biopsies displayed intercellular widening between desmosomes, a decreased number of desmosomes, and hypoplastic desmosomes in the lower epidermal layers. Acantholysis was present in two of five N+ biopsies, but only in the upper epidermal layers. The lesional skin biopsies displayed acantholysis in the higher epidermal layers. Hypoplastic desmosomes were partially (pseudo-half-desmosomes) or completely torn off from the opposing cell.
Conclusion We propose the following mechanism for acantholysis in PF: initially PF IgG causes a depletion of non-junctional Dsg1, leading to intercellular widening between desmosomes starting in the lower layers and spreading upwards. Depletion of non-junctional Dsg1 impairs the assembly of desmosomes, resulting in hypoplastic and a decreased number of desmosomes. In addition antibodies might promote disassembly of desmosomes. In the upper layers of the epidermis, where Dsg3 is not expressed and cannot compensate for Dsg1 loss, ongoing depletion of Dsg1 will finally result in a total disappearance of desmosomes and subsequent acantholysis.
Background: Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are potentially fatal blistering diseases caused by autoantibodies targeting desmoglein adhesion proteins. Previous studies have shown an IgG4>IgG1 predominance of anti-desmoglein antibodies in pemphigus; however, no studies have examined total serum IgG4 levels in pemphigus. IgG4 is induced by chronic antigen stimulation, which could occur with persistent skin blistering and potentially elevate the total serum IgG4 relative to other IgG subclasses in pemphigus patients.
Objectives: The primary aim of the study was to quantitate total and desmoglein-specific IgG subclasses in pemphigus patients.
Methods: IgG subclasses and desmoglein-specific IgG1 and IgG4 were quantitated in PV, PF, and age-matched normal sera using a subclass ELISA. The effectiveness of IgG4 depletion in blocking PV IgG pathogenicity was determined using a keratinocyte dissociation assay.
Results: Desmoglein-specific antibodies comprised a median of 7.1% and 4.2% of total IgG4 in PV and PF patients, with 8-fold and 4-fold enrichment in IgG4 versus IgG1. Total serum IgG4, but not other IgG subclasses, was enriched in PV and PF patients compared to age-matched controls (p=0.004 and p=0.005, respectively). IgG4 depletion of PV sera reduced pathogenicity in a keratinocyte dissociation assay and showed that affinity-purified IgG4 is more pathogenic than other serum IgG fractions.
Conclusions: Desmoglein-specific autoantibodies are significantly enriched in IgG4, which may explain the enrichment of total serum IgG4 in some pemphigus patients. By preferentially targeting autoimmune rather than beneficial immune antibodies, IgG4-targeted therapies may offer safer treatment options for pemphigus.
IgA pemphigus includes subcorneal pustular dermatosis (SPD)-type and intraepidermal neutrophilic IgA dermatosis (IEN)-type. Cases of IgG/IgA pemphigus have recently been documented1. Nonetheless, individual reports of IgA pemphigus indicate considerable heterogeneity.
Erythema multiforme (EM) is an uncommon, immune-mediated disorder that presents with cutaneous or mucosal lesions or both. In herpes simplex virus (HSV)–associated EM, the findings are thought to result from cell-mediated immune reaction against viral antigen-positive cells that contain the HSV DNA polymerase gene (pol ). The target lesion, with concentric zones of color change, represents the characteristic cutaneous finding seen in this disorder. Although EM can be induced by various factors, HSV infection continues to be the most common inciting factor. Histopathologic testing and other laboratory investigations may be used to confirm the diagnosis of EM and to differentiate it from other clinical imitators. Imitators of EM include urticaria, Stevens-Johnson syndrome, fixed drug eruption, bullous pemphigoid, paraneoplastic pemphigus, Sweet’s syndrome, Rowell’s syndrome, polymorphus light eruption, and cutaneous small-vessel vasculitis. Because disease severity and mucosal involvement differ among patients, treatment should be tailored to each patient, with careful consideration of treatment risk vs benefit. Mild cutaneous involvement of EM can be managed primarily with a goal of achieving symptomatic improvement; however, patients with HSV-associated recurrent EM and idiopathic recurrent EM require treatment with antiviral prophylaxis. Inpatient hospitalization may be required for patients with severe mucosal involvement that causes poor oral intake and subsequent fluid and electrolyte imbalance. With this review, we strive to provide guidance to the practicing dermatologist in the evaluation and treatment of a patient with EM.