Vesiculobullous Disorders Overview

A common subepidermal blistering disease affecting older adults and is frequently associated with neurological issues.

Autoantibodies mainly against BP180 and BP230 are characteristic of this disease.

A blistering disease affecting pregnant women, often starting during the second trimester.

This disease is characterized by pruritic urticarial plaques and tense bullae, often flaring around delivery or during menstruation.

Anti-BP180 antibodies trigger the pathogenic mechanisms of Bullous Pemphigoid.

This disease can be triggered by certain medications, highlighting the importance of considering medication history.

HLA-DQB1*0301 is associated with increased risk of developing Bullous Pemphigoid.

Bullous Pemphigoid often presents with tense blisters on normal or erythematous skin and may involve the oral mucosa.

Autoimmune blistering disease targeting desmoglein 3, a protein that holds skin cells together.

A sign of pemphigus vulgaris where a gentle pressure on the skin causes blistering due to the loss of cell-to-cell adhesion.

A sign of pemphigus vulgaris where a gentle pressure on the mucous membrane causes blistering due to the loss of cell-to-cell adhesion.

A test where a patient's serum is exposed to a monkey esophagus, and a fluorescent dye is used to detect antibodies against desmoglein 3. This helps diagnose pemphigus vulgaris.

A blood test that measures the amount of antibodies against desmoglein 3 in the blood. This helps monitor disease activity in pemphigus vulgaris.

A method of preparing a sample of skin for microscopic examination. This helps diagnose pemphigus vulgaris.

A technique used to detect antibodies against desmoglein 3 in the skin. This helps diagnose pemphigus vulgaris.

A type of pemphigus where the antibodies target desmoglein 1, the protein that holds cells in the outer layer of the skin together. This results in scaly, red lesions mainly on the skin.

Immune system mistakenly targets the basement membrane of the skin, causing separation and blister formation.

Commonly observed in elderly individuals, and symptoms include large fluid-filled blisters, often starting near the umbilicus.

A type of skin biopsy that involves staining with hematoxylin and eosin. Used to examine tissue structure.

A test that uses fluorescent antibodies to detect specific antibodies in the blood. Used to diagnose Bullous Pemphigoid.

First-line treatment for mild to moderate Bullous Pemphigoid involves topical corticosteroids.

Second-line treatments for Bullous Pemphigoid include oral steroids, immunosuppressants, and intravenous immunoglobulin (IVIg).

A chronic skin condition that causes itchy, blistering, and papular eruptions. Commonly associated with Celiac disease.

A specific type of antibody found in the blood of most people with Dermatitis Herpetiformis and Celiac Disease.

A type of antibody that can be tested for in the blood, but not always present in people with Dermatitis Herpetiformis.

A type of antibody specific to the skin condition Dermatitis Herpetiformis, detected in the blood.

A medication used to treat the skin symptoms of Dermatitis Herpetiformis. It provides quick relief from itching.

A vital step in managing Dermatitis Herpetiformis. It often takes several months to see improvements.

The gold standard for diagnosing Dermatitis Herpetiformis. It involves examining a skin sample under a microscope.

The most common site for Dermatitis Herpetiformis lesions, often seen on the elbows, knees, and back.

Oral corticosteroid therapy (usually prednisone) is the standard of care for Pemphigus Vulgaris, typically in doses around 1 mg/kg per day.

Immunosuppressants like mycophenolate, azathioprine, or cyclophosphamide are often used to reduce steroid dependence and maintain remission in Pemphigus Vulgaris. These drugs help control the immune system's attack on skin cells.

Rituximab, an antibody that targets B-cells, is a standard initial treatment for moderate to severe Pemphigus Vulgaris when the disease is refractory (doesn't respond to other treatments), rapidly progressing, or a threat to organ function. It often works alongside oral steroids.

Pemphigus Vulgaris is a serious disease. Before the advent of prednisone, people with this condition had a life expectancy of less than 5 years. Now, thanks to effective treatments, the mortality rate is much lower (5-15%).

Pemphigus Vulgaris is the most common form of pemphigus, but there are other types as well. These include Pemphigus vegetans, Pemphigus foliaceus, Pemphigus erythematosus, Fogo selvagem, drug-induced pemphigus, paraneoplastic pemphigus, and IgA pemphigus.

Pemphigus vegetans is a unique variant of Pemphigus Vulgaris characterized by vegetating plaques, often in the groin and axillae.

Fogo selvagem is a type of Pemphigus foliaceus that predominantly occurs in Brazil.