Blistering Disorders: Bullous Pemphigoid, Pemphigus and Emergencies

In Malay: Penyakit kulit melepuh

Autoimmune blistering disorders are uncommon conditions in which the immune system attacks the proteins that hold skin together, causing blisters and raw areas; the main types are bullous pemphigoid, which mostly affects older adults, and pemphigus vulgaris, which often begins with mouth sores. Sudden widespread blistering can also signal an emergency such as Stevens-Johnson syndrome. A consultant dermatologist such as Dr Kartini Farah Rahim in Shah Alam, Malaysia, can confirm the diagnosis with a skin biopsy and manage long-term treatment.

At a glance

Main types Bullous pemphigoid and pemphigus vulgaris, with several rarer forms
Bullous pemphigoid Mostly affects older adults, usually aged 70 and over
Pemphigus vulgaris clue Mouth sores occur in 50 to 70% of patients and are often the first sign
Contagious No. Autoimmune blistering diseases are not infections
Key test Skin biopsy with direct immunofluorescence, plus blood tests
Emergency sign Widespread blistering or peeling with sores in the mouth or eyes, often after a new medicine
Malay term Kulit melepuh (lepuh means blister)

When to see a doctor

  • EMERGENCY: widespread blistering or peeling skin, especially with sores in the mouth, eyes or genitals. Go to the emergency department.
  • EMERGENCY: a flu-like illness followed by a painful, spreading rash, particularly within weeks of starting a new medicine. Go to the emergency department.
  • EMERGENCY: swelling of the lips, tongue or throat, or difficulty breathing. Call 999.
  • Blisters that appear without a clear cause, or keep coming back
  • An older person with persistent itch or an eczema-like rash that is not responding to usual treatment
  • Mouth ulcers lasting longer than three weeks
  • Signs of infection in blistered skin, such as pus, spreading redness or fever

A blister is a pocket of fluid that forms when layers of skin separate. Most blisters come from friction, burns, insect bites or infections. A smaller group of conditions, the autoimmune blistering diseases, happen when the body’s own immune system attacks the skin. These are uncommon, can be serious, and usually need specialist diagnosis and long-term care.

What causes blisters on the skin?

Blisters can be caused by:

  • friction, burns and sunburn
  • insect bites and plant reactions
  • infections such as impetigo, chickenpox, shingles (kayap) and hand, foot and mouth disease (see skin infections)
  • eczema and allergic contact dermatitis (see skin allergies)
  • reactions to medicines
  • autoimmune diseases such as bullous pemphigoid and pemphigus
  • rare inherited conditions that make skin fragile from birth

Blisters that appear without an obvious cause, keep returning, or come with mouth sores deserve a medical assessment.

When are blisters a medical emergency?

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are rare but life-threatening reactions, usually to medicines. DermNet describes them as variants of the same condition, differing in how much skin detaches: under 10% of the body surface in SJS and over 30% in TEN.

Medicines most often involved include antibiotics, some anticonvulsants, anti-inflammatory painkillers and some gout medicines. DermNet notes that SJS/TEN usually develops within the first week of antibiotic therapy, but up to 2 months after starting an anticonvulsant.

The pattern often begins with a flu-like illness, such as fever, sore throat and aching, followed days later by a painful rash that blisters and peels. Sores in the mouth, eyes and genitals are common. DermNet reports mortality of up to 10% for SJS and at least 30% for TEN, which is why early hospital care matters.

Go to the emergency department immediately if you have:

  • widespread blistering or peeling skin
  • painful sores in the mouth, eyes or genitals with a rash
  • fever or a flu-like illness followed by a painful, spreading rash, especially after starting a new medicine
  • swelling of the lips, tongue or throat, or difficulty breathing (call 999)

What is bullous pemphigoid?

Bullous pemphigoid is an autoimmune blistering disease that mainly affects older people. The British Association of Dermatologists notes that it usually affects people aged 70 and over, and it occurs in men and women equally.

Typical features include:

  • severe itch, often the first symptom
  • an early phase lasting weeks, with an eczema-like or hive-like rash before blisters form
  • large, tense blisters filled with clear or blood-stained fluid, often on the limbs, skin folds and trunk
  • crusted raw areas where blisters have burst
  • mouth involvement, which is uncommon

Pemphigoid is not contagious, not caused by infection or allergy, and not hereditary. Some medicines have been linked with it, including a class of diabetes medicines (DPP-4 inhibitors) and cancer immunotherapy. According to DermNet, treatment is usually needed for several years, but in many cases pemphigoid eventually clears and treatment can be stopped.

What is pemphigus vulgaris?

Pemphigus vulgaris usually begins between the ages of 30 and 60. DermNet reports that it accounts for about 70% of pemphigus cases worldwide, and that the mouth is involved in 50 to 70% of patients.

Painful mouth ulcers that do not heal are often the first sign, so people may see a dentist or doctor for mouth problems before any skin blisters appear. The skin blisters are thin-walled and break easily, leaving painful raw areas, especially on the scalp, face, chest and back. The throat can also be affected, causing hoarseness or difficulty swallowing.

Pemphigus is a serious condition, but the American Academy of Dermatology notes that it can nearly always be brought under control with proper medical care and regular follow-up.

What other blistering diseases are there?

Condition Who is typically affected Blister pattern Mouth involved?
Bullous pemphigoid Older adults, usually over 70 Large, tense, very itchy blisters Uncommon
Pemphigus vulgaris Adults, usually aged 30 to 60 Fragile blisters that break into raw areas Often
Pemphigus foliaceus Adults Superficial, scaly, crusted patches No
Mucous membrane pemphigoid Mainly older adults Mouth, eyes and genitals; risk of scarring Yes
Dermatitis herpetiformis Adults with gluten sensitivity (coeliac disease) Very itchy small blisters on elbows, knees and buttocks Rarely
Linear IgA disease Children and adults Clusters of blisters, sometimes ring-shaped Sometimes
Pemphigoid gestationis Pregnancy Itchy blisters, often starting around the navel Rarely

How are blistering disorders diagnosed?

Because many conditions cause blisters, diagnosis usually combines:

  • a skin biopsy from the edge of a fresh blister, to see the level at which the skin splits
  • direct immunofluorescence on a second small sample of nearby skin, to show antibodies attacking the skin
  • blood tests for circulating antibodies
  • baseline health checks, such as blood count, kidney and liver function, blood sugar and blood pressure, before starting immune-suppressing treatment

People with eye, throat or genital involvement may also need input from other specialists.

How are autoimmune blistering diseases treated?

Treatment is individualised, depending on the diagnosis, how widespread it is, age and other health conditions. Options include:

  • potent topical corticosteroids, particularly for localised pemphigoid
  • oral corticosteroids to bring blistering under control
  • tetracycline-class antibiotics, used for their anti-inflammatory effect
  • steroid-sparing immunosuppressant medicines, to reduce long-term steroid exposure
  • biologic therapy for severe or resistant disease
  • skin care: gentle cleansing, non-stick dressings, and draining large blisters with a sterile needle while leaving the roof in place

Safety notes: long-term steroid tablets need monitoring of blood sugar, blood pressure and bone health. Do not stop steroid tablets suddenly. Immune-suppressing medicines increase infection risk, so report fever or spreading redness promptly.

What should people in Malaysia know about blistering disorders?

  • Delayed diagnosis: early pemphigoid can look like eczema, scabies or insect bites, and early pemphigus can look like ordinary mouth ulcers. Itch or sores that persist despite treatment should be reviewed.
  • Heat and humidity: sweat and friction make raw skin more uncomfortable and prone to infection. Loose cotton clothing and a cool environment help.
  • Diabetes medicines: diabetes is common in Malaysia. An older person taking diabetes medicines who develops persistent itch and blisters should have their medicines reviewed, but must not stop them without their doctor’s advice.
  • Traditional remedies and unregulated creams: herbal pastes, oils and powders applied to raw skin can cause irritation or infection, and some unregistered creams contain undeclared steroids.
  • Fasting and medicines: if you plan to fast during Ramadan while taking steroids or immunosuppressants, discuss timing and safety with your doctor beforehand.
  • Carers: many people with pemphigoid are elderly and rely on family members, who may need practical guidance on dressings and recognising infection.

Seeing Dr Kartini for blistering disorders

Blistering disorders are one of the clinical focus areas of Dr Kartini Farah Rahim, Consultant Dermatologist at Avisena Specialist Hospital, Shah Alam. Her training includes internal medicine as well as dermatology.

A consultation typically includes:

  • History: when the itch or blisters began, all current and recent medicines, other health conditions, and any mouth, eye or swallowing symptoms.
  • Examination: of the skin and, where relevant, the mouth, eyes and genital area.
  • Tests: a skin biopsy with direct immunofluorescence, blood tests, and baseline checks before treatment.
  • A management plan: medicines chosen to balance disease control with side effects, blister and wound care advice, and referral to other specialists where needed.
  • Follow-up: regular reviews to monitor response and side effects and to reduce treatment gradually when it is safe to do so.

If you have widespread blistering with mouth or eye sores, or feel very unwell, go to the emergency department rather than waiting for a clinic appointment. For non-urgent assessment, see appointments.

Frequently asked questions

Is bullous pemphigoid contagious?

No. Bullous pemphigoid is an autoimmune condition, meaning the immune system mistakenly attacks the skin. It is not caused by an infection or an allergy, cannot be passed to family members or carers, and is not considered hereditary. Carers can safely help with dressings and skin care, using normal hygiene measures to protect raw skin from infection.

Can bullous pemphigoid be cured?

There is no single cure, but pemphigoid can usually be controlled with treatment. The British Association of Dermatologists notes that it often settles on its own after one to five years, although some people need long-term low-dose treatment. The aim is to stop new blisters with the lowest effective treatment, then reduce medicines gradually under supervision.

Should I pop my blisters?

Do not peel off blister roofs, because the skin covering acts as a natural dressing. Large, tense blisters that are uncomfortable are sometimes drained with a sterile needle, leaving the roof in place, but ask your doctor or nurse how to do this safely. Keep raw areas clean and covered with non-stick dressings, and watch for signs of infection.

Why do I need a skin biopsy?

Many conditions cause blisters, and they look similar on the surface. A biopsy shows the level in the skin where the blister forms, and direct immunofluorescence reveals the pattern of antibodies attacking the skin. Together with blood tests, this distinguishes pemphigoid from pemphigus and other conditions, which matters because treatment and outlook differ.

Can medicines cause blistering skin diseases?

Yes. Some medicines have been linked with bullous pemphigoid, including certain diabetes medicines and cancer immunotherapy. Severe drug reactions such as Stevens-Johnson syndrome are most often triggered by antibiotics, some anticonvulsants, anti-inflammatory painkillers and some gout medicines. Never stop a prescribed medicine without advice, but seek emergency help for blistering with mouth or eye sores.

What is the difference between pemphigus and pemphigoid?

In pemphigoid, antibodies target the junction beneath the outer skin layer, producing deep, tense blisters that usually stay intact, mainly in older adults. In pemphigus, antibodies target the connections between skin cells higher up, so blisters are fragile and break easily, and mouth sores are common. Pemphigus vulgaris typically starts between ages 30 and 60 and generally needs more intensive treatment.

Are long-term steroid tablets safe for blistering diseases?

Oral steroids are often needed to bring blistering under control, but long-term use can affect blood sugar, blood pressure, bones, mood and infection risk. For this reason, doctors aim for the lowest effective dose, add steroid-sparing medicines, and arrange monitoring and bone protection. Never stop steroid tablets suddenly; if you cannot take them, contact your doctor urgently.

Sources and further reading

  1. Bullous pemphigoid (DermNet)
  2. Pemphigoid (also known as bullous pemphigoid) (British Association of Dermatologists)
  3. Pemphigus vulgaris (DermNet)
  4. Pemphigus: Overview (American Academy of Dermatology)
  5. Stevens-Johnson syndrome/toxic epidermal necrolysis (DermNet)
  6. Stevens-Johnson syndrome (NHS)
Medical information notice. This page offers general education written for patients in Malaysia. It is not a diagnosis or a substitute for a consultation with a qualified doctor who can examine you. If you have severe symptoms, such as swelling of the lips, tongue or throat, difficulty breathing, widespread blistering or a rapidly spreading painful rash with fever, go to the nearest emergency department or call 999. Read our editorial and medical review policy.

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Dr Kartini sees adults and children at Avisena Specialist Hospital and Avisena Women’s & Children’s Specialist Hospital in Shah Alam. Please WhatsApp first to confirm her schedule.