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

• Senear-Usher Syndrome

What is Pemphigus Erythematosus? (Definition/Background Information)

• Pemphigus Erythematosus is an uncommon and localized form of pemphigus foliaceus, a blistering skin disorder, which mostly affects older adults. It is characterized by superficial erosions with crust and scaly skin formation
• Pemphigus Erythematosus does not present mucosal involvement. The condition is also known as Senear-Usher Syndrome, and it presents similar features as systemic lupus erythematosus (SLE), a different autoimmune disorder.
• Pemphigus is a benign and chronic skin disorder commonly affecting mid-to-elderly adults. It is characterized by the formation of painful blisters or erosions on the surface of the skin and mucous membranes, and is caused by immune system dysregulation
• The treatment of choice for Pemphigus Erythematosus is topical and oral corticosteroids. With prompt and appropriate therapy, the outcomes are usually good, and no long-term complications are noted

Who gets Pemphigus Erythematosus? (Age and Sex Distribution)

• Pemphigus Erythematosus may be observed in individuals of all age groups. However, they are most common in the elderly adults
• Both males and females are affected, and no gender predilection is seen
• Worldwide, the condition may affect individuals of all racial and ethnic groups

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

• The risk factors for Pemphigus Erythematosus are not well-defined
• A positive family history of pemphigus foliaceus may increase one’s risk for Pemphigus Erythematosus
• Exposure to sunlight may trigger the skin condition in some individuals

An association with myasthenia gravis and thymoma (carcinoma of the thymus gland) is noted in rare cases; however, the cause-effect relationship between these conditions and Pemphigus Erythematosus is not well-established.

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 Erythematosus? (Etiology)

The exact cause of development of Pemphigus Erythematosus is not well-understood. Research experts inform that it may develop due to a combination of genetic and environmental factors. The condition presents features similar to systemic/cutaneous lupus erythematosus, an autoimmune disorder.

• Pemphigus Erythematosus 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 Erythematosus 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 Erythematosus?

The signs and symptoms of Pemphigus Erythematosus may include:

• A butterfly type rash on the face (cheeks) associated with redness and swelling (termed malar eruption). This feature is also noted in some cases of lupus
• The skin lesions can accumulate fluid to become blisters, which then break to form ulcers and erosions
• The blisters involve the superficial skin layers and may rupture when rubbed, called the Nikolsky's sign
• Following rupture and discharge of fluid, the sores erode with the formation of crusts and scaly skin
• The condition may progress over many weeks to involve other regions of the body
• The mucosal surfaces are generally not affected

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

How is Pemphigus Erythematosus Diagnosed?

A diagnosis of Pemphigus Erythematosus 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 is fragile enough to peel off.
• 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, and culture of the fluid being drained may be undertaken, as needed, to check for any infections
• 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. This may include:

• Cutaneous lupus erythematosus
• Fogo selvagem
• Impetigo
• Systemic lupus erythematosus

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 Erythematosus?

The possible complications due to Pemphigus Erythematosus include:

• Severe emotional stress
• Cosmetic issues
• Superimposed bacterial and fungal skin infections
• In some individuals, the condition may persist for a long period
• Overall decreased quality of life
• Treatment complications and medication side effects

How is Pemphigus Erythematosus Treated?

Early treatment is important in preventing extensive skin involvement by Pemphigus Erythematosus 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 Erythematosus be Prevented?

It may not be possible to currently prevent Pemphigus Erythematosus. 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

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

• The prognosis of Pemphigus Erythematosus is generally good with prompt and adequate treatment. However, in some individuals, the condition may persist for extended durations
• 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 Erythematosus:

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

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