Alles Wat U Moet Weten Over Pemphigus Vulgaris - Symptomen, Behandeling en Meer

Hoe ontstaat het?

Pemfigus ontstaat door een autoimmuun reactie, dat is een reactie van het lichaam tegen het eigen weefsel. Bij pemfigus is deze reactie gericht tegen bepaalde bestanddelen van de opperhuid, waardoor de opperhuid plaatselijk loslaat en blaren gaat vormen.
Waarom de autoimmuun reactie ontstaat is nog niet bekend. In sommige gevallen wordt het veroorzaakt door geneesmiddelen (penicillamine, captopril).
Bij pemfigus vulgaris zijn de blaren vrij slap en met vocht gevuld. De blaren gaan door stoten of krabben makkelijk stuk.
De blaren bij pemphigus foliaceus zijn heel slap en gaan al heel snel stuk zodat er al snel oppervlakkige wondjes (erosies) ontstaan.

Als pemphigus vulgaris onbehandeld wordt gelaten is het een levensbedreigende aandoening, pemphigus foliaceus heeft onbehandeld een veel gunstiger beloop.
Sinds de ontdekking van prednison is pemphigus echter goed te behandelen. Door prednison wordt de autoimmuunreactie onderdrukt en blijft de opperhuid intact.
Meestal wordt prednison gecombineerd met azathioprine (dat ook de afweer verlaagt) zodat de dosering prednison niet te hoog hoeft te zijn.
Meestal zal uw arts ervoor kiezen de behandeling te beginnen met een relatief hoge dosering prednison. Zodra er geen nieuwe blaren meer ontstaan wordt de dosering stapje voor stapje verlaagd.

Behandeling pemfigus vulgaris:

0 prednison gecombineerd met azathioprine (Imuran ®) of
0 prednison alleen

indien deze therapie niet aanslaat:

0 goudinjecties
0 dapson
0 ciclosporine

Behandeling pemfigus foliaceus:

0 lokale corticosteroiden (hormooncrèmes)
0 prednison gecombineerd met azathioprine of
0 prednison alleen

Behandeling pemfigus van de mond:

0 lokale corticosteroiden (speciale preparaten voor gebruik in de mond)
0 bij pijn bij eten: lidocaine lokaal

What is the outcome for someone with pemphigus?

Today, the outcome looks good. For most people, the disease can be controlled with treatment. Many can eventually stop their treatment for a while.

Before medicines like prednisone and azathioprine were used to treat pemphigus, a person lived about 5 years after getting pemphigus vulgaris, the most common type. Thanks to medicines and other treatments, this has changed. Few people die of pemphigus.

People still have to deal with other problems that pemphigus can cause. The medicines can cause serious side effects.

Other problems include taking the time to see doctors and get treatment. To keep pemphigus under control, you may need to see both a dermatologist and your primary care physician. If pemphigus affects your eyes, you’ll also need to see an eye doctor. You may also see a dentist for help with pemphigus or a doctor who specializes in the ears, nose, and throat.

Serious weight loss is another problem that pemphigus can cause.

These problems can be managed with help from your dermatologist and other doctors.

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References
Amagai M. “Pemphigus.” In: Bolognia JL, et al. Dermatology. (second edition). Mosby Elsevier, Spain, 2008:417-29.

Craythorne EE Mufti G, et al. “Rituximab used as a first-line single agent in the treatment of pemphigus vulgaris.” J Am Acad Dermatol. 2011 Nov,65(5):1064-5.

Huang A, Madan RK et al. “Future therapies for pemphigus vulgaris: rituximab and beyond.” J Am Acad Dermatol. 2016 Apr,74(4):746-53.

National Institutes of Health. “Questions and answers about pemphigus.” Last updated June 2015. Last accessed August 18, 2016.

News Medical. “Two new studies find potential genetic cause and new treatment method for autoimmune diseases.” July 13, 2016. Last accessed August 16, 2017.

Stanley JR. “Pemphigus.” In: Wolff K, Goldsmith LA, et al. Fitzpatrick’s Dermatology in General Medicine (seventh edition). McGraw Hill Medical, New York, 2008: 459-74.

Who is at Risk for Developing this Disease?

PV has been associated with both myasthenia gravis and thymoma. It is more common for patients with thymoma to have pemphigus than for patients with pemphigus to have thymoma.

Penicillamine and captopril have been associated with PV. The prevalence of pemphigus (including vulgaris and foliaceus) in penicillamine users is estimated at 7%. Although angiotensin-converting enzyme inhibitors other than captopril are not as strongly associated with pemphigus, it is reasonable to change these medications. Diet has also been proposed as a risk factor for PV (eg, garlic, onions). However, discontinuation of penicillamine or other medications and modification of diet rarely result in disease remission.

Neonatal PV results from placental transfer of anti-desmoglein antibodies from mother to newborn. Disease severity can range from mild to fatal, usually correlating with disease severity in the mother. Disease remits as maternal antibody is catabolized (usually complete by 6 months).

Introduction

Pemphigus vulgaris (PV) is an autoimmune disease that results in blisters on cutaneous and mucosal surfaces and is characterized primarily by acantholysis.[1] Pemphigus is derived from pemphix, the Greek word for blister. Pemphigus was first described in 1788 by Stephen Dickson, who observed a patient with a blister on her tongue.[2] Although PV is not contagious as initially thought, there have been possible triggers identified that might induce PV in patients with other autoimmune disorders.[1]

Exposure to certain medications like penicillamine and captopril can trigger PV. Such a trigger can happen through the effects on binding to molecules involved in cell adhesion, influence on enzymes that mediate keratinocyte aggregation, and by stimulating neoantigen formation.[13] Furthermore, nonsteroidal antiinflammatory agents, penicillin, and cephalosporins have also been associated with drug-induced PV.[13][14] Finally, controversial case reports associating PV with certain foods like red wine, garlic, leek, and peppers exist, though such an association is not supported by robust evidence.[15]