Lichen Planus Pemphigoid
This page covers the following:
What is lichen planus pemphigoid?
Lichen planus pemphigoid is a combination of an inflammatory disease of undetermined cause (lichen planus) and an autoimmune bullous disease (pemphigoid-like).
In the vast majority of cases, pemphigoid lichen planus is not a serious disease. The severity depends on the mucous membranes affected and the extent of the lesions: erosive genital involvement, as well as eye, throat and oesophagus lesions, can be severe.
Very rarely, in certain forms of lichen, cancer may occur locally on old lesions.
What are the symptoms of lichen planus pemphigoid?
Symptoms combine those of lichen planus with those of bullous pemphigoid or of mucous membrane pemphigoid.
Presentation can vary widely, depending on the type of lichen planus (cutaneous and/or oral and/or genital), the type of associated pemphigoid and the areas affected. Blisters do not systematically occur in the same area as lichen lesions and may be far apart (e.g. lichen on the wrists and blisters on the back, or cutaneous lichen and oral blisters). Lichen lesions usually precede the appearance of blisters, sometimes by several years.
What causes lichen planus?
The causes of this immune system dysfunction are not yet well understood. It is not known why these two diseases can occur together in the same patient. According to some hypotheses, lichen planus favours the appearance of autoantibodies directed against the skin or mucous membranes; according to others, lichen planus is already an autoimmune disease.
Pemphigoid lichen planus can affect people of all ages, ethnic origins and genders. It is not contagious, and is not hereditary.
How does it evolve?
The disease usually progresses in successive flare-ups. It can be severe in the absence of treatment. The trigger may differ according to whether they occur after lichen or bullous lesions.
How will I be treated?
There are effective treatments which generally combine topical corticosteroid treatment for lichen lesions with general treatment for bullous disease.
Treatment generally takes place in two phases: initial treatment, usually involving high doses of medication to halt disease progression and heal lesions and then a maintenance treatment to maintain remission, the duration of which is discussed on a case-by-case basis.
Since these diseases can affect several mucous membranes, their management requires the involvement of several specialists - the dermatologist generally coordinates care and, depending on the case, enlists the help of other experts. Depending on the type of damage (e.g. eye or laryngeal lesions), the choice of medication needs to be adapted.
Treatment of bullous disease follows the approaches discussed on our bullous pemphigoid or mucous membrane pemphigoid pages. In general, when your disease is under control, your daily life can be virtually unchanged.
Where can I find further information?
DermNet NZ are a very good source of information on this and other skin diseases. The British Association of Dermatologists have a Patient Information Leaflet.
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