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Pemphigus-Types, Diagnosis, and Treatment

Published on May 12, 2022   -  4 min read

Abstract

Pemphigus is a skin disorder characterized by oral and mucocutaneous lesions with increased mortality risk. Read the article below to know more about this condition.

Contents

Introduction:

The term pemphigus originates from the Greek word ‘pemphix,’ which means blister or bubble. Therefore, this may be definable as a group of chronic blistering diseases of the skin or the epithelial layers. Pemphigus disease mainly occurs due to the production of IgG autoantibodies against the domains of cell membrane proteins, especially cells called keratinocytes resulting in the loss of cellular adhesion (the phenomenon of acantholysis).

What Are the Types of Pemphigus?

Pemphigus has three major subtypes:

A. Pemphigus Vulgaris: in this subtype, the blisters develop deep in the epidermal or oral epithelium above the basal layer when observed histologically. Pemphigus Vulgaris clinically manifests as a mucosally dominant variety with multiple mucocutaneous lesions that start in the oral mucosa classically. These lesions further extend to the skin. In the oral mucosa, the manifestation of flaccid blisters and prone to rupture are characteristic signs of this disease. These lesions can be painful, erosive, and occur more frequently in the buccal mucosal region. Weight loss and malnutrition are other physical features seen with the onset of the disease. Apart from the oropharyngeal mucosal disease, other mucous membranes are involved, including the lining of the larynx, esophagus, conjunctiva, nose, genitalia, anus, etc. Lesions of the skin may extend to or involve the head, upper trunk, and groin regions accompanied by crusted erosive lesions or erythematous skin, or both.

B. Pemphigus Foliaceus: In this subtype, the pemphigus foliaceus blisters occur in the superficial layers of the epidermis. In pemphigus foliaceus, it not only affects mucosal areas. Patients may clinically present with multiple scaly and crusted erosive lesions that are flaky or seen as circumscribed patches on the skin.

C. Paraneoplastic Pemphigus is different from the previous two subtypes by the presence of underlying or associated neoplasms. Mostly these neoplasms may be of lymphoid tissue origin.

What Are the Etiology and Pathogenesis of Pemphigus?

The onset and etiology of this disease are linked to the age group, usually in the fifth to sixth decade of life. Women are more affected than men comparatively in the incidence groups observed generally. Most patients who manifest clinical symptoms are usually between 50 to 60 years of age. Increased prevalence of low titers of disease-associated serum autoantibodies can be traced according to research and documented evidence in first-degree relatives of some patients suffering from pemphigus. This proves that there is a possibility of definite genetic transmission or linkage. HLA alleles are also identified as risk factors for genetic transmission, but the correlation between specific HLA genes and the patient’s clinical features remains elusive.

However, other factors apart from genetics that would trigger the potential onset of pemphigus like environmental determinants, drugs containing a thiol group or metal chelating agents like Penicillamine, other medications like Captopril, etc., have been hypothesized to interfere with the biochemistry of the keratinocyte membrane mechanism causing a disturbance of immune balance. This phenomenon, in turn, leads to the promotion of acantholysis that causes pemphigus.

Other environmental factors researched as triggers for disease onset are viral infections due to herpes simplex virus, dietary factors, physiological or psychological stressor mechanisms.

How to Diagnose Pemphigus Vulgaris?

On physical examination, apart from the clinical features, patients manifest positive Nikolsky signs in the active phase of the disease. Therefore, the lesional biopsy is a reliable method to detect pemphigus Vulgaris.

Histopathologically a ‘tombstone effect’ can be observed microscopically, which is the presence of residual basal keratinocytes at the basement membrane zone, which might be seen on the floor of the blister.

Direct immunofluorescence microscopy of lesional biopsy specimens obtained would prove a reliable and sensitive diagnostic test for detecting any three forms of pemphigus.

The desmoglein ELISA test is particular in sensitivity and specificity for detecting pemphigus. Patients suffering from pemphigus have circulating anti-desmoglein antibodies that do not occur otherwise in typically healthy individuals. Western blotting or immunoprecipitation of epithelial cells and other methods like obtaining keratinocyte extracts may not be practical in diagnosing pemphigus vulgaris and pemphigus foliaceus but are often used to identify non-desmoglein target proteins in patients suffering from the third subtype, i.e., the paraneoplastic pemphigus lesions.

What Are the Differential Diagnoses of Pemphigus?

  1. Autoimmune Diseases: bullous pemphigoid, mucous membrane pemphigoid, lichen planus, epidermolysis bullosa, bullous dermatosis, bullous lupus erythematosus, etc.

  2. Underlying Disease or Syndrome: aphthous stomatitis, erythema multiforme, Steven-Johnson syndrome, toxic epidermal necrolysis /TENS syndrome, lichen planus, Grover disease, seborrheic dermatitis, pustular dermatosis.

  3. Infectious Diseases: staphylococcal infections, bullous impetigo, acute herpetic stomatitis, etc.

  4. Certain Genetic Diseases.

How to Manage Pemphigus?

The following are all other adjuvant or accessory medications, techniques, and therapies that would be instituted by the physician based upon the mortality risk and extent of pemphigus infection in the affected individual. It includes:

Osteoporosis counseling is also provided if the corticosteroid treatment lasts for the patient for more than three months to counteract the issue of bone resorption and fragility.

The reported mortality risk due to the side effects of long-term pemphigus treatment includes respiratory tract infections, life-threatening sepsis or septicemia, cardiovascular disease, and peptic ulcer disease.

Conclusion:

To conclude, pemphigus vulgaris and pemphigus foliaceus are more associated with risks of morbidity and mortality. However, mortality has now, in recent decades, decreased due to the advancements in therapeutic and regular use of corticosteroids and adjuvant immunosuppressants based on timely diagnosis and treatment planning by the physician.

Frequently Asked Questions


1.

Where Does Pemphigus Usually Start?

Pemphigus is a severe skin condition and is classified into different types. Regarding pemphigus vulgaris, the blisters usually start in the mouth and then on the skin and genital mucosa. These blisters may cause discomfort while eating and swallowing food. In the case of pemphigus foliaceus, the blisters do not occur in the mouth and are most commonly noted on the chest, shoulders, and back. These blisters are itchy and painful.

2.

Is Pemphigus Treatable?

Pemphigus causes fluid-filled bumps on the skin and mucous membrane. The doctor may suggest the following medications to treat it:

- Corticosteroids - Topical or oral corticosteroids reduce inflammation.

- Immunosuppressive drugs like Cyclophosphamide and Mycophenolate reduce the immune response; thus, the attack on healthy cells is prevented.

- Intravenous immunoglobulin therapy aids in destroying the antibodies that caused pemphigus.

3.

Are Pemphigus and Pemphigoid the Same?

No, pemphigus and pemphigoid are not the same. However, here are the differentiating features of both:

- Pemphigus - It causes lesions and bumps on the outer skin. These fluid-filled blisters burst quickly.

- Pemphigoid - It causes firm blisters on the lower layers of skin and does not break easily.

Both lesions are diagnosed by skin biopsy and immunofluorescence microscopy. In addition, the dermatologist may suggest corticosteroids to manage pemphigus and pemphigoid lesions.

4.

How Does Pemphigus Appear?

Pemphigus is a chronic skin disorder that leads to fluid-filled bumps on the skin and mucous membrane. It mainly affects middle-aged or older individuals. The fluid-filled blisters are fragile and rupture easily. The characteristics of lesions vary according to their types.

- In pemphigus erythematosus, the lesion looks red and crusty.

- In pemphigus vulgaris, the blisters are painful and appear on the mouth, skin, ad mucous membrane.

- In pemphigus vegetans, wart-like lesions appear.

5.

How Many Types of Pemphigus Are There?

Pemphigus is an autoimmune skin disorder that results in pus-filled blisters on the skin and mucous membrane. The different types of pemphigus are as follows:

- Pemphigus vulgaris - Painful blisters may occur more commonly in the mouth. It may also occur in the skin and mucous membrane.

- Pemphigus foliaceus - Itchy blisters may occur on the upper layer of the skin, and the mucous membrane is mostly spared.

- Pemphigus vegetans - Wart-like blisters form on the skin-fold areas like the armpit and groin. It is type of pemphigus vulgaris.

- Drug-induced pemphigus - Specific medications like antibiotics and antihypertensives may cause blisters.

- Paraneoplastic pemphigus - Lesions may develop over the lips, mucous membrane, and skin. They occur with tumors in the body and may resolve if the tumor is removed.

6.

What Is Pemphigus Vegetans?

Pemphigus vegetans is rare variant of pemphigus vulgaris. It accounts for 1 % to 2 % of all pemphigus. It mainly occurs as a result of antibodies against a specific protein that is responsible for binding cells. It leads to blisters and bumps on the trunk, legs, and arms. It may occur in the mouth, nasal, and vaginal regions. The lesions look cauliflower-like, and they may rupture and ulcerate. The diagnosis is made by direct immunofluorescence microscopy.

7.

Can I Live Long With Pemphigus?

Pemphigus is a chronic skin condition that causes painful blisters and sores on the skin.

Corticosteroids and immunosuppressive drugs have shown good improvement in patients with pemphigus. In addition, the use of such medications has improved the survival rate also. However, certain medications may worsen the condition. Therefore, it is essential to discuss with the dermatologist regarding drugs. Therefore, to lead a better life and to prevent the worsening of pemphigus, the following measures can be followed:

- Avoid excessive sun exposure.

- Take care of your skin and prevent injuries.

- Do not stress yourself.

- Avoid infections.

- Eat soft and bland foods if you have mouth sores.

8.

Is Pemphigus Serious?

Pemphigus is a severe skin disorder, and it remains lifelong. It may lead to life-threatening conditions if left untreated. However, medications like corticosteroids and immunosuppressants have improved the symptoms. But the long-term treatment of pemphigus causes the following complications:

- Cardiovascular disease.

- Respiratory tract infections.

- Peptic ulcer.

- Spread infection to the bloodstream.

9.

Does Antibody Cause Pemphigus?

Pemphigus is a rare autoimmune disorder that is mainly caused by autoantibodies. Specific proteins called desmosomal adhesion proteins (desmogleins 1 and 3) are necessary for maintaining the structure of the upper layer of the skin. In pemphigus, antibodies may develop against these proteins, thus separating the epidermis cells. It thereby results in the formation of blisters and ulcers on the skin. It is usually detected by removing a tissue sample through biopsy and viewing it under immunofluorescence.

10.

How Do I Know About Oral Pemphigus?

Pemphigus may result in blisters on the skin and oral and genital mucous membranes. But, in many individuals, oral lesions appear first, and they may also develop in 80 % to 90 % of individuals with pemphigus vulgaris. Therefore, the early treatment of these oral lesions may limit the occurrence of skin lesions. The commonly affected regions in the oral cavity are:

- Buccal mucosa.

- Palatal mucosa.

- Lingual and labial mucosa.

- Gingiva.

The doctor may suggest oral corticosteroid, painkiller, and mouth wash to get relief.

11.

How Does Pemphigoid on the Skin Appear?

Pemphigoid is an autoimmune skin disorder that affects the lower layer of the skin. It results in the formation of firm blisters that do not break easily. The blisters usually develop over the flexed surface of the extremities, lower abdomen, and groin. It is of two types, namely:

- Bullous pemphigoid - The blisters extensively develop on the skin and do not occur on mucous membranes. The commonly affected sites are arms, legs, back, and groin.

- Gestational pemphigoid - The blisters develop during the second trimester of pregnancy and on the navel area, which later spreads to other body areas.

12.

How Does a Rheumatologist Treat Pemphigus Vulgaris?

Rheumatoid arthritis is a chronic condition that causes inflammation and pain in joints. Individuals with rheumatoid arthritis undergo treatment with disease-modifying anti-rheumatic drugs (DMARDs) like D-penicillamine. Several reports show that long-term treatment with such medications may cause drug-induced pemphigus. These drugs may break the link between cells of the epidermis, thus causing blisters. The rheumatologist may consider providing alternative drugs to prevent the worsening of drug-induced pemphigus.

Last reviewed at:
12 May 2022  -  4 min read

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