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

• LP Pemphigoides
• LPP (Lichen Planus Pemphigoides)

What is Lichen Planus Pemphigoides? (Definition/Background Information)

• Lichen Planus Pemphigoides (LPP) is a rare cutaneous condition that presents features of two distinct skin conditions, namely lichen planus and bullous pemphigoid
  • Lichen planus (LP) is a common benign condition of generally unknown cause affecting the skin and mucus membranes in adults
  • Bullous pemphigoid is an uncommon skin disease mainly affecting the elderly population, characterized by the formation of blisters on the skin
• The cause of Lichen Planus Pemphigoides is not well understood. It is characterized by the formation of painful blisters on the surface of the skin and mucous membranes. The skin also turns thick and leathery
• The treatment of Lichen Planus Pemphigoides may involve topical and oral corticosteroids. With prompt and appropriate therapy, the outcomes may be good. However, this may vary from one individual to another depending on its severity

Who gets Lichen Planus Pemphigoides? (Age and Sex Distribution)

• Lichen Planus Pemphigoides is a rare condition affecting the skin
• The condition typically affects middle-aged men (between 40 and 50 years of age), although females can be affected as well
• The presentation of symptoms may occur at any age, and the condition has been reported in children as well
• Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for Lichen Planus Pemphigoides? (Predisposing Factors)

The risk factors that may predispose an individual to develop Lichen Planus Pemphigoides may include:

• Use of medications that include:
• Paracetamol
• Ibuprofen
• Angiotensin-converting enzyme (ACE) inhibitors (for controlling blood pressure)
• Anti-tuberculosis medications
• Certain statins (such as simvastatin, used for lowering cholesterol)
• Cinnarizine (a calcium channel blocker and anti-histamine)
• Psoralen and ultraviolet A light therapy (PUVA therapy)
• Narrowband ultraviolet B (UVB) phototherapy

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 Lichen Planus Pemphigoides? (Etiology)

The exact cause of Lichen Planus Pemphigoides is not known.

• The cause may be associated with allergies or an immune response
• Certain medications and therapies may increase the probability of developing this condition

What are the Signs and Symptoms of Lichen Planus Pemphigoides?

The signs and symptoms of Lichen Planus Pemphigoides may vary among affected individuals in type and severity and may include:

• Presence of itchy skin blisters
• The blisters may even form in the mouth (oral mucosal surfaces)
• Nail abnormalities
• Thickening of skin
• Rashes on skin that are lighter than the surrounding skin
• Eye abnormalities that may include:
  • Inflammation of the eyelids and conjunctival membrane of the eye
  • Eye irritation and redness
  • Excess tearing

How is Lichen Planus Pemphigoides Diagnosed?

Lichen Planus Pemphigoides is diagnosed on the basis of the following information:

• Physical examination of the individual and medical history evaluation
• 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 microscope findings, the pathologist arrives at a definitive diagnosis. A separate specimen is taken and stained for disease-causing antibodies detectable in the specimen in an immunofluorescent biopsy

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 Lichen Planus Pemphigoides?

The complications of Lichen Planus Pemphigoides may include:

• Severe scarring
• Emotional stress from cosmetic concerns
• Low self-esteem
• Superimposed bacterial and fungal skin infections

Complications may occur with or without treatment and in some cases, due to treatment.

How is Lichen Planus Pemphigoides Treated?

The treatment for Lichen Planus Pemphigoides is determined by the severity of signs and symptoms in an affected individual and may include one or a combination of the following:

• Topical steroids
• Oral steroid medications
• Antibiotics such as doxycycline
• Nicotinamide (vitamin B3)
• Vitamin A or retinoids such as isotretinoin or acitretin
• Immune-suppressing agents such as cyclosporine A

Regular medical screening at periodic intervals with tests and physical examinations is recommended.

How can Lichen Planus Pemphigoides be Prevented?

Lichen Planus Pemphigoides may be preventable, or at least the severity of its signs and symptoms reduced, by identifying the trigger(s) for the condition.

What is the Prognosis of Lichen Planus Pemphigoides? (Outcomes/Resolutions)

• The prognosis of Lichen Planus Pemphigoides is dependent upon the severity of the signs and symptoms and associated complications, if any
• Individuals with mild conditions have a better prognosis than those with severe symptoms and complications. Typically, the prognosis may be assessed on a case-by-case basis

Additional and Relevant Useful Information for Lichen Planus Pemphigoides:

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

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