What are the other Names for this Condition? (Also known as/Synonyms)

• Pemphigus Foliaceous
• PF (Pemphigus Foliaceus)

What is Pemphigus Foliaceus? (Definition/Background Information)

• Pemphigus Foliaceus is a rare variant of pemphigus, a skin disorder, which is characterized by superficial blisters that erode with crust and scaly skin formation. It is typically observed in individuals with generally healthy skin, and many cases are observed in middle-aged adults
• Pemphigus is a benign and chronic skin disorder commonly affecting mid-to-elderly adults. It is characterized by the formation of painful blisters on the surface of the skin and mucous membranes, and is caused by immune system dysregulation
• The treatment of choice for Pemphigus Foliaceus is the use of topical and oral corticosteroids. With prompt and appropriate therapy, the outcomes are usually good, and no long-term complications are noted. However, the condition can take years to resolve in some individuals

Pemphigus Foliaceus is grouped into various subtypes that include:

• Pemphigus erythematosus
• Pemphigus herpetiformis
• Endemic Pemphigus Foliaceus (fogo selvagem)
• Pemphigus sebhorrhoicus
• IgA Pemphigus Foliaceus
• Paraneoplastic Pemphigus Foliaceus
• Drug-induced Pemphigus Foliaceus

Who gets Pemphigus Foliaceus? (Age and Sex Distribution)

• Pemphigus Foliaceus may be observed in individuals of all age groups. However, they are most common in the sixth decade (between 50 and 60 years)
• Both males and females are affected, and no gender predilection is seen
• Worldwide, the condition may affect individuals of all racial and ethnic groups. However, these may vary based on the specific form of Pemphigus Foliaceus

What are the Risk Factors for Pemphigus Foliaceus? (Predisposing Factors)

• The risk factors for Pemphigus Foliaceus are dependent on the individual subtypes
• A positive family history of Pemphigus Foliaceus may increase one’s risk for the same
• It is informed that an exposure to sunlight may trigger the skin condition in some individuals
• Use of certain medications is also a risk factor for the condition

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Pemphigus Foliaceus? (Etiology)

The exact cause of development of Pemphigus Foliaceus is not well-understood. Research experts inform that it may develop due to a combination of genetic and environmental factors.

• Pemphigus Foliaceus is described as an autoimmune blistering disorder of skin in which an individual's immune system attacks their own skin components, due to the production of immunoglobulin antibodies. This results in the characteristic signs and symptoms of the disorder
• Generally, antibodies are produced in the body to kill microorganisms that are harmful to the body. But, in pemphigus, the antibodies attack the healthy skin and mucous membranes
• These antibodies act against the proteins in the skin, known as desmogleins, which play a role in skin cell adhesion. When these proteins are attacked, the skin cells get separated from each other, causing fluid accumulation between the skin layers. This leads to the formation of blisters

Pemphigus Foliaceus is not a contagious skin condition. It cannot be transmitted from one individual to another through direct physical contact or sharing of materials.

What are the Signs and Symptoms of Pemphigus Foliaceus?

The signs and symptoms of Pemphigus Foliaceus may include:

• Presence of small fluid-filled blisters on skin; the blisters involve the superficial skin layers
• The blisters may rupture or extend when rubbed, called the Nikolsky's sign
• Following rupture (the blisters rupture easily) and discharge of fluid, the sores erode with the formation of crusts and scaly skin
• The condition may progress over many weeks, affecting the skin on the shoulders, chest, and upper back
• In severe cases, or when early medical intervention is not sought, the entire torso and the neck, face, and scalp regions may be affected
• The mucosal surfaces are generally not affected in Pemphigus Foliaceus

Pain is generally minimal, but individuals may feel burning sensation, irritation, and itchiness.

How is Pemphigus Foliaceus Diagnosed?

A diagnosis of Pemphigus Foliaceus may involve the following exams and procedures:

• Physical examination of the individual and medical history evaluation
• Nikolsky sign: The physician may rub the skin near the blister to check if the skin peels-off. If it does, it is a sign indicating the likelihood of pemphigus (as well as some other conditions)
• Blood tests: Blood tests are conducted to check the level of desmoglein antibodies in blood. An elevated level of this antibody can help confirm the presence of pemphigus
• Blood tests, analysis of pus, culture of the fluid being drained, to check for any infections, if needed
• Dermoscopy: Dermoscopy is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
• Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using ultraviolet light. It is performed to examine the change in skin pigmentation
• Skin biopsy: A skin biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis
• Direct and indirect immunofluorescence studies on the skin biopsy specimen

A differential diagnosis may be necessary to eliminate other conditions that present similar signs and symptoms.

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Pemphigus Foliaceus?

The possible complications due to Pemphigus Foliaceus include:

• Severe emotional stress and discomfort
• Cosmetic issues
• Superimposed bacterial and fungal skin infections
• In some individuals, the condition may take many years to resolve
• Decreased quality of life
• Treatment complications and medication side effects

How is Pemphigus Foliaceus Treated?

Early treatment is important in preventing extensive skin involvement by Pemphigus Foliaceus and avoiding superimposed skin infections. The treatment measures available may include:

• Topical and systemic corticosteroids are the mainstay of therapy, mainly for mild to moderate cases
• If necessary, systemic immunosuppressant medications may be prescribed for severe cases
• Undertaking treatment of any secondary infections through administration of antibiotic or antifungal agents
• Good skin care and general body hygiene is important

Regular follow up visits with the healthcare provider is important and recommended.

How can Pemphigus Foliaceus be Prevented?

It may not be currently possible to prevent Pemphigus Foliaceus. This also depends on the specific subtype of the condition. However, the risk for the same may be decreased by considering some of the following steps:

• Avoiding known triggers
• Minimizing sun exposure
• If there is a family history of the condition, then genetic counseling will help assess risks before planning for a child
• When possible, the offending medication (causing Pemphigus Foliaceus) may be discontinued and/or alternatives prescribed

What is the Prognosis of Pemphigus Foliaceus? (Outcomes/Resolutions)

• The prognosis of Pemphigus Foliaceus is generally good with prompt and adequate treatment. However, in some individuals, the condition may persist for several years
• An early appropriate treatment can help avoid long-term complications and ensure faster healing. In some, a spontaneous resolution of the condition is also noted

Additional and Relevant Useful Information for Pemphigus Foliaceus:

The following DoveMed website link is a useful resource for additional information:

https://www.dovemed.com/healthy-living/skin-disorders/