Life Threatening Skin Rash series – Pemphigus Vulgaris

Pemphigus vulgaris eczema

Pemphigus Vulgaris (PV) is a rare, auto-immune disease that mistakes skin cells and mucous membranes as foreign matters and attacks them. This results in blisters and sores of the skin and the mucous membranes. It is more common in middle-aged and older people. PV is not infectious.

What Causes Pemphigus Vulgaris?

Pemphigus vulgaris is caused by an increase in the desmoglein antibodies where these antibodies incorrectly binds to protein desmoglein 3, which is found in desmosomes in the keratinocytes near the bottom of the epidermis. It then results in a separation of the skin layer and formation of blisters. The triggers of this faulty immune systems are not fully known though genetics play a part. 

Types of Pemphigus Vulgaris

Mucosal PV: Only the mucous membranes are affected, but not the skin. This may include the mucous membranes lining the mouth, nose, throat and genitals.

Mucocutaneous PV: Both the mucous membranes and skin are affected.

Symptoms of Pemphigus Vulgaris

  • Fever, Chills
  • Muscle aches
  • Rash, first appearing in mouth and then to rest of the skin
  • Blisters
  • Raw, moist, tender skin
  • Peeling Skin 
  • Fluid loss
  • Pain

Diagnosis is usually made by a skin specialist because it is uncommon, thus not often seen by general practitioners. Physical examination and lesion biopsy (including immunofluorescence to assess level of antibodies) are used to diagnose PV. Early treatment helps to prevent PV from being widespread.

Stages of Pemphigus Vulgaris

  1. Formation of blisters and sores around the mouth
  2. Bursting of mouth blisters, similar to ulcers
  3. More widespread blisters at other parts of skin with oozing, crusting and peeling of skin.
  4. Separation of outer layer skin with gentle touch (“Nikolsky’s sign”)
  5. Recovery of raw skin (6 to 8 weeks)

Treatment of Pemphigus Vulgaris

There is no treatment that specifically cures PV but instead treatment is to reduce blistering and limit flare-up. This is often via immunosuppressant therapy. Oral corticosteroids and immunosuppressive drugs may be prescribed. In more severe cases, it can be via intravenous methylprednisolone and cyclophosphamide (Pulsed therapy) which can last from 6 months to a year. Dosages are typically higher at the start of treatment and gradually reduces when the condition is stable (without flare-up). 

For severe cases, the patient will be hospitalized in the burn unit as the skin requires similar care to burns. Apart from immunosuppressant therapy, treatment also encompasses (i) pain relief, (ii) anesthetic lozenges to reduce mouth ulcer pain, (IiI) prevention of dehydration through intravenous drip, (Iv) medication to prevent bacterial or fungal infection

Care of the Skin

Care of the skin includes application of lotions and wet dressings (wound care, wet compress). Also minimize contact sports to avoid skin trauma.

Mouth Blisters and Care

If blisters occur in the voice box (larynx), the voice will be hoarse. If the blisters occur in the gullet, swallowing is painful. Where mouth blisters make it difficult to eat, supplements may be prescribed. Steroid, antiseptic or anesthetic mouth wash may also be prescribed for the mouth blisters. Avoid foods that may irritate the inside of mouth such as spicy, acidic or hard foods.

Pemphigus Vulgaris and Eczema

PV is sometimes diagnosed late as mistaken for dyshidrotic eczema or pompholyx which also has blisters. However, dyshidrotic eczema is not life threatening and believed to be due to stress or allergies.

Complications of Pemphigus Vulgaris

It is a difficult condition to treat as it is an autoimmune disease that has no cure. The mortality rate is 10%. There can also be complications associated with long-term use of oral corticosteroids and immunosuppressants. Other complications are secondary skin infection, dehydration and sepsis (bloodstream infection).

Anyone have had PV? Do share in the comments, thanks loads for spreading encouragement and experience!

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