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

• Hirata Disease
• Insulin Autoimmune Hypoglycemia

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

• Lichen Planus Pigmentosus (LPP) is a rare form of lichen planus. It is characterized by oval or irregularly-shaped brown to gray-brown macules and patches on the skin. Areas that are exposed to sun such as the forehead, temples and neck are most commonly affected. However, the macules and patches may also develop on the trunk or in places where two areas of skin touch or rub together (i.e. the armpit, groin, etc)
• Lichen Planus Pigmentosus is a chronic, relapsing condition with periods of worsening symptoms separated by periods of remission (decreasing or disappearing symptoms)
• The cause of Lichen Planus Pigmentosus is unknown, but studies suggest it may be triggered by UV light, viral infections, or agents applied to the skin such as mustard oil and amla oil
• Treatment for Lichen Planus Pigmentosus depends on the symptoms in each person

(Source: Lichen Planus Pigmentosus; Genetic and Rare Disease Information Center (GARD) of National Center for Advancing Translational Science (NCATS), USA.)

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

• Lichen Planus Pigmentosus is a rare disorder of unknown prevalence
• The presentation of symptoms typically occrs in adulthood
• Both males and females may be affected
• The condition is prevalent among dark-skinned individuals, such as people of Indian, Middle Eastern, and Latin-American origin

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

The risk factors for Lichen Planus Pigmentosus may include:

• Henna-based dyes used for skin/hair
• Use of mustard or gooseberry (amla) oil on skin/hair
• Viral infections (hepatitis C virus)
• Bazex syndrome (a rare 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 Lichen Planus Pigmentosus? (Etiology)

The cause of Lichen Planus Pigmentosus is not known at the present time.

• Certain dyes (such as henna), oils (mustard or amla), and viral infections are known to predispose an individual to the condition
• In addition, an association of Lichen Planus Pigmentosus with Bazex syndrome has also been reported

What are the Signs and Symptoms of Lichen Planus Pigmentosus?

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

• Oval or irregularly-shaped brown to gray-brown macules and patches on the skin, in areas that are exposed to sun such as the forehead
• However, the macules and patches may also develop on the trunk or in places where two areas of skin touch or rub together (i.e. the armpit, groin, etc)

(Source: Lichen Planus Pigmentosus; Genetic and Rare Disease Information Center (GARD) of National Center for Advancing Translational Science (NCATS), USA.)

How is Lichen Planus Pigmentosus Diagnosed?

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

• Complete physical examination
• Thorough medical history evaluation
• Assessment of signs and symptoms
• Laboratory tests
• Imaging studies
• Skin biopsy studies for confirmation

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 Pigmentosus?

The complications of Lichen Planus Pigmentosus may include:

• Scarring
• Recurrence of condition after it resolves

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

How is Lichen Planus Pigmentosus Treated?

There is currently no consensus about treatment for Lichen Planus Pigmentosus. Management includes avoiding things that make it worse in each case, such as sun exposure or friction in body folds (via weight loss or wearing loose clothing)

Treatment options may include:

• Vitamin A
• Topical corticosteroids
• Topical calcineurin inhibitors such as tacrolimus (medications that are typically used to treat eczema)
• Dapsone (a medication used to treat leprosy and skin infections)
• Skin lightening agents
• Laser therapy

(Source: Lichen Planus Pigmentosus; Genetic and Rare Disease Information Center (GARD) of National Center for Advancing Translational Science (NCATS), USA.)

How can Lichen Planus Pigmentosus be Prevented?

The exact cause of Lichen Planus Pigmentosus is not known, and therefore, no preventive methods or guidelines are available at the present time.

• Identifying the triggers for the development of this condition may help prevent or reduce the signs and symptoms
• Active research is currently being performed to explore the possibilities for treatment and prevention of disorders such as Lichen Planus Pigmentosus

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

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

• The prognosis of Lichen Planus Pigmentosus is reported to be good, since the symptoms generally resolve on their own or with treatment
• However, the condition is chronic, with recurrence of signs and symptoms even after treatment and resolution
• Affected individuals may experience periods of worsening and improving signs and symptoms

Additional and Relevant Useful Information for Lichen Planus Pigmentosus:

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

http://www.dovemed.com/diseases-conditions/rare-disorders/