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

• PH (Pemphigus Herpetiformis)

What is Pemphigus Herpetiformis? (Definition/Background Information)

• Pemphigus Herpetiformis (PH) is a rare subtype of pemphigus foliaceus, a skin disorder, which presents clusters of pus/fluid filled blisters resembling herpes (a viral infection causing blisters)
• In Pemphigus Herpetiformis, the skin lesions are arranged in linear or curving arrays and clusters, like the herpes virus infections. The condition may be observed in children and 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 Herpetiformis is topical and oral corticosteroids. With prompt and appropriate therapy, the outcomes are usually good, and no long-term complications are noted. However, the condition may take long duration to resolve in some individuals

Who gets Pemphigus Herpetiformis? (Age and Sex Distribution)

• Pemphigus Herpetiformis may be observed in individuals of all age groups. It represents between 6-7% of all pemphigus foliaceus cases
• 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 Herpetiformis? (Predisposing Factors)

• A positive family history of Pemphigus Herpetiformis may increase one’s risk for the same
• It is informed that an exposure to sunlight may trigger the skin condition in some individuals

An association with the following conditions have been occasionally noted with Pemphigus Herpetiformis:

• Autoimmune hemolytic anemia
• Esophageal cancer
• Prostate cancer
• Psoriasis
• Systemic lupus erythematosus (SLE)

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 Herpetiformis? (Etiology)

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

• Pemphigus Herpetiformis 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 Herpetiformis 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 Herpetiformis?

The signs and symptoms of Pemphigus Herpetiformis may include:

• Presence of pus or fluid filled blisters on skin - these are termed pustules or vesicles
• Some skin lesions appear as plaques, which are lesions that are red and elevated skin surface about 1 cm in diameter
• The skin lesions involve the superficial skin layers
• The arrangement of skin lesions are in clusters that give it an appearance of the infectious condition herpes. These may be circular, cyclical, or gyrate (spiral)
• The blisters may rupture 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 several areas of the body, but commonly the trunk
• The mucosal surfaces are generally not affected

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

How is Pemphigus Herpetiformis Diagnosed?

A diagnosis of Pemphigus Herpetiformis 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,
• 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.

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 Herpetiformis?

The possible complications due to Pemphigus Herpetiformis include:

• Severe emotional stress
• Cosmetic issues
• Superimposed bacterial and fungal skin infections
• Some cases of Pemphigus Herpetiformis are known to progress to pemphigus vulgaris, a more severe form of pemphigus
• In some individuals, the condition may take many years to heal
• Overall decreased quality of life
• Treatment complications and medication side effects

How is Pemphigus Herpetiformis Treated?

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

It may not be possible to currently prevent Pemphigus Herpetiformis. 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 Herpetiformis? (Outcomes/Resolutions)

• The prognosis of Pemphigus Herpetiformis is generally good with prompt and adequate treatment. However, in some individuals, the condition may persist for a long duration
• An early appropriate treatment can help avoid long-term complications and ensure faster healing

Additional and Relevant Useful Information for Pemphigus Herpetiformis:

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

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