INTRODUCTION 

Individual cells of the skin and mucosa, the keratinocytes, are anchored to one another, resist mechanical trauma, prevent microorganisms from entering the body, and protect against fluid loss.

In pemphigus patients, an autoimmune process disrupts the desmosome’s function, leading to a breakdown of cutaneous and mucosal barriers.

HISTORICAL BACKGROUND 

The modern concept of pemphigus divides it into variants: pemphigus vulgaris, pemphigus vegetans, pemphigus foliaceus, pemphigus erythematosus (Senear-Usher syndrome). 

The changes in the theory of pemphigus over time have been re-examined. The word “pemphigus” was given by Boissier de Sauvages. He stated pemphigus major is an acute, febrile, blistering disorder that remains only two weeks. 

Wichmann was the first to describe pemphigus as a chronic bullous disease; he used the term, febris bullosa, to describe bullous eruptions of short duration. Unfortunately, few studies accepted Wichmann's more restricted theory, and universal dermatologists continued to use the word pemphigus for the various vesicular or bullous disorders. Willan had a much more different opinion of the disease.  

Recent evidence 

Recent evidence indicates that there are two phenotypes of PV, mucosal dominant and mucocutaneous, which are possibly shifting from one form to the other form with time.

Classification of Pemphigus 

1. vulgaris
2. vegetans
3. erythematosus
4. foliaceus

PEMPHIGUS VULGARIS 

EPIDEMIOLOGY 

PV is an uncommon disease with an annual incidence of 1 to 5 per million populations per year. It has been commonly seen in the fourth to sixth decades of life.

PATHOPHYSIOLOGY 

The initiation of PV is not known, still, some evidence says that the epithelial breakdown in PV is mediated by autoantibodies of the IgG type.

CLINICAL PRESENTATION 

PV affects the oral mucosa and the skin, leading to superficial blisters and chronic ulcer formation.

INVESTIGATION 

Laboratory test 

A biopsy specimen studied by both routine histopathological and immunopathology is the basic procedure to analyse or diagnose the cause of chronic mucosal erosion and ulceration.

MANAGEMENT

Oral lesions of pemphigus Vulgaris may show effect with the application of partially topical or intralesional corticosteroids or other immunosuppressant drugs.

PEMPHIGUS VEGETANS 

INTRODUCTION 

Pemphigus Vulgaris is an autoimmune disorder characterized by the production of IgG autoantibodies against intercellular adhesion protein desmoglein, leading to acantholysis. The disorder mainly affects middle-aged people. Lesions are primarily flexural, however, vegetations may occur at any site.

CLINICAL FEATURES 

Pemphigus vegetans is an uncommon variant of pemphigus vulgaris. 

The median age of onset is 40–50 years. 

Two clinical subtypes of pemphigus vegetans exist, characterized initially by flaccid bullae and erosions (Neumann) or pustules. 

Lesions are typically located in the oral cavity.

ORAL MANIFESTATION 

Pemphigus vegetans are considerably less common in the mouth than pemphigus Vulgaris. It usually presents clinically as serpiginous ulcers that are most frequent on the tongue and lips.  

INVESTIGATION 

MICROSCOPIC EXAMINATION 

Microscopic examination.

Biopsy 

Immunofluorescent study.

MANAGEMENT 

Treatment is the same as that for PV.

PEMPHIGUS FOLIACEUS (SUPERFI CIAL PEMPHIGUS, FOGO SELVAGEM)

Pemphigus foliaceus (PF) is normally a benign kind of pemphigus. It is an autoimmune skin disease where there is a formation of superficial blisters. 

It is typified by clinical involvement of healthy-appearing skin that blisters when rubbed (the Nikolsky sign). 

It includes the following six subtypes: 

• Pemphigus erythematosus (PE).

• pemphigus herpetiformis (PH).

• Endemic pemphigus foliaceus.

• Immunoglobulin A (IgA) pemphigus foliaceus.

• paraneoplastic pemphigus foliaceus (PNPF).

• Drug-induced pemphigus foliaceus.

AETIOLOGY 

Precipitating factors include medications and ultraviolet light radiation. 

CLINICAL FEATURES

Pemphigus foliaceus is manifested by characteristic early bullous lesions which rapidly rupture and dry which is suggestive of exfoliative dermatitis or eczema. Most common in older adults but may occur in young children as well.

Brazilian pemphigus (Fogo selvagem or Brazilian wildfire) is a mild endemic form of pemphigus foliaceus found in tropical regions, particularly in Brazil, that often occurs in children and frequently in family groups. The pattern of the disease is similar to that of pemphigus foliaceus. 

Oral lesions in pemphigus foliaceus are rare, according to Perry and Brunsting

(1965) in their extensive study of this form of the disease.

TREATMENT 

Therapy for PF is usually less aggressive than that for pemphigus Vulgaris because of their lower morbidity and mortality rates. Few results indicate that nonsteroidal treatment of pemphigus is possible. 

Mestinon may be used to slow down the progression of the disease and to treat mild cases with chronic lesions in limited areas.       

PEMPHIGUS ERYTHEMATOSUS

INTRODUCTION 

In 1926, Senear and Usher described pemphigus erythematosus as a variant of pemphigus. 

EPIDEMIOLOGY 

Review shows cases of pemphigus erythematosus that occurred in the adult population, and several cases of pemphigus erythematosus in children have been reported.  A predominance of girls has been observed in patients with pemphigus erythematosus who were less than 20 years old.

CLINICAL FINDINGS 

The lesion occurs over the trunk, lower extremities, upper extremities, face, oral mucosa, soft palate, scalp, and buttocks. the lesion of the pemphigus erythematosus has been noted to be limited to the seborrheic areas of the skin. They may be present on the face, typically in a butterfly distribution over the nose and the malar regions similar to the distribution of facial lesions seen in patients with lupus erythematosus.       

IMMUNOFLUORESCENCE STUDIES 

Direct immunofluorescence studies demonstrated the presence of immunoglobulins.

Autoantibodies are demonstrated by both direct and Indirect immunofluorescence techniques in patients with each of the four types of pemphigus. 

Treatment 

Patients with pemphigus erythematosus have been treated with oral and topical corticosteroids, /3-sympatholytic agents, immunosuppressive agents, jamarson therapy, sulphapyridine, chloroquine, anticholinergic agents, or a combination of these medications.

Patients with pemphigus erythematosus have been treated with a variety of medications. Currently, corticosteroids alone or corticosteroids in combination with an immunosuppressive agent appear to be the most effective methods of treating patients with pemphigus erythematosus; however, severe side effects have occurred with these medications. 

Come and join “Project Global Cure” to be more knowledgeable about your own body system and spread this knowledge to others.