pemphigus

Pemphigus: A Rare Autoimmune Disease

Pemphigus is a group of rare autoimmune diseases that cause blisters and sores on the skin or mucous membranes, such as in the mouth or on the genitals [1]. It is characterized by the formation of painful blisters and erosions on the skin and mucous membranes, most commonly inside the mouth [6].

Types of Pemphigus

Pemphigus can affect various parts of the body, including the skin, mucous membranes, and internal organs. The disease can be classified into several types, including:

• Pemphigus vulgaris: a rare autoimmune disease that is characterized by painful blisters and erosions on the skin and mucous membranes [6].
• Pemphigoid: a group of subepidermal, blistering autoimmune diseases that primarily affect the skin, especially in the lower abdomen, groin, and other areas [3].

Causes and Symptoms

The exact cause of pemphigus is unknown, but it is believed to be an autoimmune disease, where the body's immune system attacks healthy skin cells [8]. The symptoms of pemphigus can vary depending on the type and severity of the disease. Common symptoms include:

• Blisters: painful blisters or fluid-filled bumps that form on the skin or mucous membranes.
• Erosions: open sores or ulcers that can develop from the blisters.
• Skin lesions: red, itchy, or scaly patches on the skin.

References

[1] Pemphigus is a group of rare skin disorders that cause blisters and sores on the skin or mucous membranes, such as in the mouth or on the genitals. [2] Pemphigus is a group of autoimmune skin conditions that cause sores, blisters or fluid-filled bumps to form on your skin and mucus membranes. [3] Aug 8, 2023 — Pemphigoid is a group of subepidermal, blistering autoimmune diseases that primarily affect the skin, especially in the lower abdomen, groin and ... [6] Pemphigus vulgaris is a rare autoimmune disease that is characterised by painful blisters and erosions on the skin and mucous membranes, most commonly inside ... [7] Pemphigus is a group of potentially life-threatening disorders characterized by blisters in mucous membranes and skin. [8] by M Kasperkiewicz · 2017 · Cited by 563 — Pemphigus is a group of IgG-mediated autoimmune diseases of stratified squamous epithelia, such as the skin and oral mucosa, in which acantholysis (the loss of ... [9] Sep 16, 2020 — Pemphigus vulgaris is an autoimmune, intraepithelial, blistering disease affecting the skin and mucous membranes and is mediated by circulating ...

Pemphigus Signs and Symptoms

Pemphigus is a group of autoimmune skin conditions that cause sores, blisters or fluid-filled bumps to form on your skin and mucus membranes [4]. The main symptom of pemphigus is blistering of the skin and in some cases, the mucosal surfaces, such as the inside of the mouth, nose, throat, etc. [2].

Common Symptoms:

• Blisters on otherwise healthy skin
• Blisters that burst easily and leave very sore patches
• Red, sore-looking areas on the skin and mucous membranes
• Soft and limp blisters that break open easily, ooze fluid and become open sores [8]

Specific Symptoms of Pemphigus Vulgaris:

• Pemphigus vulgaris often starts in the mouth, causing painful blisters to form that can make eating and drinking difficult [5]
• Blisters on the skin and mucous membranes that are soft and limp
• Blisters break open easily, ooze fluid and become open sores

Other Symptoms:

• The sores may become infected and ooze
• Pemphigus can develop slowly, causing blisters in the same area for years [3]

It's essential to note that pemphigus symptoms can vary from person to person, and not everyone will experience all of these symptoms. If you suspect you or someone else has pemphigus, it's crucial to consult a healthcare professional for proper diagnosis and treatment.

References: [1] Not available [2] Aug 19, 2024 — The main symptom of pemphigus is blistering of the skin and in some cases, the mucosal surfaces, such as the inside of the mouth, nose, throat, ... [3] What are the signs and symptoms of pemphigus? When someone develops pemphigus, it can develop slowly, causing blisters in the same area for years. [4] Pemphigus is a group of autoimmune skin conditions that cause sores, blisters or fluid-filled bumps to form on your skin and mucus membranes. [5] May 18, 2022 — Symptoms of pemphigus vulgaris usually arise in your mouth, which causes painful blisters to form that can make eating and drinking difficult. [6] Pemphigus vulgaris causes blisters that burst easily and leave very sore patches. Pemphigus vulgaris inside a person's mouth. There are red, sore-looking ... [7] What are the symptoms of pemphigus vulgaris? Pemphigus vulgaris often starts in the mouth. Symptoms include: Blisters on otherwise healthy skin; Blisters ... [8] What are the symptoms of pemphigus? · Blisters on skin and mucous membranes that are soft and limp. · Blisters break open easily, ooze fluid and become open sores ...

Diagnostic Tests for Pemphigus

Pemphigus, a group of autoimmune diseases characterized by blistering of the skin and mucous membranes, can be diagnosed through various tests.

• Physical Exam: A dermatologist will examine the blisters, sores, and crusts to assess their appearance and location. [2]
• Medical History: The dermatologist will also take a detailed medical history to understand any previous conditions or allergies that may be relevant to the diagnosis. [2]
• Blood Tests: Blood tests can help detect and identify antibodies in your blood that are known to cause pemphigus. These tests include Indirect Immunofluorescence (IIF) and Enzyme-Linked Immunosorbent Assay (ELISA). [1][3][4]
• Biopsy Studies: A biopsy of perilesional skin or mucosa using direct immunofluorescence is considered a gold standard for diagnosing pemphigus. This test can detect in vivo deposits of antibodies and other immune complexes. [5][8]
• Skin Biopsy with Direct and Indirect Immunofluorescence: A skin biopsy followed by direct and indirect immunofluorescence testing can also be used to diagnose pemphigus. [7]

Diagnostic Criteria

A diagnosis of pemphigus requires a combination of clinical presentation, histopathology consistent with pemphigus, and either a positive direct immunofluorescence (DIF) microscopy or a positive ELISA test. [9][10]

These diagnostic tests can help healthcare providers accurately diagnose pemphigus and develop an effective treatment plan.

References: [1] Context 1 [2] Context 2 [3] Context 3 [4] Context 4 [5] Context 5 [7] Context 7 [8] Context 8 [9] Context 9 [10] Context 10

Treatment Options for Pemphigus

Pemphigus, an autoimmune disease characterized by blistering skin and mucous membranes, can be effectively managed with various drug treatments. The primary goal of treatment is to ease symptoms, prevent new blisters, and control the disease.

• Steroids: Systemic prednisone in combination with adjuvant immunosuppressants is a common treatment approach for pemphigus (Apr 24, 2023). Topical steroids can also be used to address oral erosion and skin lesions.
• Immunosuppressive Agents: Adjuvant immunosuppressants are often prescribed alongside steroids to help control the immune system's attack on healthy skin cells. Examples include dapsone, intravenous immunoglobulin, and rituximab-pvvr (1).
• Biologics: More recently, biologics have been used to treat pemphigus, targeting specific immune messages and interrupting the signal that leads to blister formation (12).
• Glucocorticoid-Sparing Agents: Mycophenolate mofetil is commonly used as a first-line glucocorticoid-sparing agent for moderate-to-severe pemphigus vulgaris (11).

Treatment Plan

A treatment plan for pemphigus may involve one or more of the following:

• Topical steroids to address oral erosion and skin lesions
• Systemic prednisone in combination with adjuvant immunosuppressants
• Biologics to target specific immune messages
• Glucocorticoid-sparing agents like mycophenolate mofetil

Important Considerations

• Treatment for pemphigus takes time before results are seen, and new blisters may stop forming after several weeks (10).
• Mild pemphigus treatment involves lower steroid doses compared to moderate/severe disease treatment.
• Biologic response modifiers can be effective in controlling the disease but require careful dosing due to their potency.

References:

[1] Treatment for pemphigus usually starts with medicines to ease symptoms and prevent new blisters. These may include steroids and medicines that target the immune system. ... If first line medicines aren't helping you, your healthcare professional may suggest another drug, such as dapsone, intravenous immunoglobulin or rituximab-pvvr ...

[4] Apr 24, 2023 — Pemphigus is usually treated with systemic prednisone in combination with adjuvant immunosuppressants.

[6] Mycophenolate mofetil is commonly used as a first-line glucocorticoid-sparing agent for the treatment of moderate-to-severe pemphigus vulgaris and is recommended in treatment guidelines. 2-4

[12] Treatment of Pemphigus. There is no cure for pemphigus, but treatment can control the disease in most people. ... Because they are potent drugs, your doctor will prescribe the lowest dose possible to achieve the desired benefit. Biologic response modifiers (or biologics). These target specific immune messages and interrupt the signal, helping ...

[13] Drug-induced pemphigus can occur as PF or PV secondary to medication use. Drug-induced pemphigus can arise days to months following initiation of medication. ... Generally, mild pemphigus treatment involves lower steroid doses compared to moderate/severe disease treatment, which involves higher steroid doses with the addition of steroid-sparing ...

[15] How is pemphigus vulgaris treated? Treatment for pemphigus vulgaris focuses on managing symptoms and preventing complications like infections. Treatment is unique to each person and could include: Taking medicines to treat infections. Stopping any medicines that cause symptoms. Using medicines, creams or ointments to treat, soothe and heal sores.

Differential Diagnoses of Pemphigus

Pemphigus, a group of life-threatening blistering disorders, requires accurate diagnosis to differentiate it from other conditions. The differential diagnoses for pemphigus include:

• Aphthous stomatitis: A condition characterized by recurrent ulcers in the oral cavity.
• Lichen planus: An inflammatory skin disease that can cause blisters and erosions.
• Oral candidiasis: A fungal infection of the mouth that can cause white patches or blisters.
• Pemphigoid: A group of autoimmune diseases characterized by blistering of the skin and mucous membranes.
• Bullous impetigo: A bacterial infection of the skin that causes blisters.
• Subcorneal pustular dermatosis: A rare skin condition characterized by pus-filled blisters.
• Subacute cutaneous lupus erythematosus: A type of lupus that can cause blistering of the skin.
• Allergic contact dermatitis: An allergic reaction to a substance that can cause blisters and skin irritation.
• Bullous systemic lupus erythematosus (BSLE): A rare form of lupus that can cause blistering of the skin.
• Dermatologic manifestations of glucagonoma: A rare tumor that can cause skin symptoms, including blistering.

These conditions can be identified through clinical findings, immunofluorescence tests, histopathological examination, and periodic acid-Schiff test using smear samples. Accurate diagnosis is crucial to provide appropriate treatment for pemphigus patients.

References:

• Context 1, 11, 13, 14, 15