Bullous pemphigoid is a blistering skin disease which usually affects middle aged or elderly persons. It is an immunobullous disease, i.e. the blisters are due an immune reaction within the skin. Childhood bullous pemphigoid is rare.
What does bullous pemphigoid look like?
Bullous pemphigoid results in crops of tense, fluid-filled, itchy bullae (large blisters). The blisters may be filled with clear, cloudy or blood-stained fluid. They may arise from normal-looking or inflamed skin.
A nonspecific red, itchy rash may be present for several weeks or months before blisters appear. The rash may be thought to be due to dermatitis or urticaria. The early plaques may resolve temporarily or persist, with or without treatment. They may have a smooth or dry surface and are often ring-shaped (annular).
Bullous pemphigoid may be localized to one area, or widespread on the trunk and proximal limbs. Frequently it affects the skin around skin folds. In severe cases, there may be blisters over the entire skin surface as well as blisters inside the mouth.
Bullous pemphigoid without blisters
Some patients have a diagnosis of bullous pemphigoid made despite not having any bullae. This has been called nonbullous pemphigoid and can affect any body site. Symptoms may include:
- Severe itch without skin lesions (apart from scratch marks)
- Eczema (itchy dry red patches)
- Urticated erythema (itchy smooth red patches)
- Nodular prurigo (itchy lumps)
How is the diagnosis of pemphigoid made?
When typical bullae are present, a dermatologist can often make the diagnosis clinically. In most cases the diagnosis will be confirmed by a skin biopsy of a typical blister. Under the microscope, the pathologist can see a split between the outside layer of the skin, the epidermis, and the inside layer, the dermis. See pathology of bullous pemphigoid. Direct immunofluorescence staining highlights antibodies along the basement membrane that lies between the epidermis and dermis. Indirect immunofluorescence test on blood serum may find circulating pemphigoid antibodies.
In patients with nonbullous pemphigoid, the diagnosis is made by a positive direct or indirect immunofluorescence test.
What causes bullous pemphigoid?
Bullous pemphigoid is thought to occur because IgG immunoglobulins (antibodies) and activated T lymphocytes (white blood cells) attack components of the basement membrane, particularly a protein known as the BP antigen BP180, or less frequently BP230. These proteins are within the NC16A domain of collagen XVII. BP180 is also called Type XVII collagen. These are associated with the hemidesmosomes, structures that ensure the epidermal keratinocyte cells stick to the dermis to make a waterproof seal. The ‘autoimmune’ reaction to these proteins can be thought of as a type of allergy to one's own skin.
In many patients, skin antibodies can also be detected circulating in the blood stream (positive indirect immunofluorescence).
Several studies have shown bullous pemphigoid to be more prevalent in patients that have had neurological disease, particularly stroke, dementia and Parkinson disease.
What is the treatment?
If the pemphigoid is very widespread, hospital admission may be advised so the blisters and raw areas can be expertly dressed. Antibiotics may be required for secondary bacterial infection.
Most patients with bullous pemphigoid are treated with steroid tablets, usually prednisone. The dose is adjusted until the blisters have stopped appearing, which usually takes several weeks. The dose of prednisone is then slowly reduced over many months or years. As systemic steroids have many undesirable side effects, other medications are added to ensure the lowest possible dose (aiming for 5 to 10mg prednisone daily). These other medications may include:
- Topical steroids (usually clobetasol propionate); these may be used as sole treatment in mild or localised disease
- Tetracycline antibiotics
- High dose intravenous immunoglobulin
Recently, rituximab has been found to help in severe cases resistant to standard therapy.
Treatment is usually needed for several years. In most cases the pemphigoid eventually completely clears up and the treatment can be stopped.
If you have pemphigoid, make sure you understand your treatment – do not alter it without consulting your dermatologist or general practitioner.
Assessment and monitoring
As systemic treatment may be required for long periods, the extent and severity of the disease should be recorded carefully at baseline and at follow-up appointments. The following aspects may be considered.
- Body sites affected (skin and mucous membranes)
- Type of lesion: transient and nontransient
- Numbers of lesions: blisters, urticarial weals, eczematous plaques,
- Severity of itch (pruritus)
- Observation point: initial phase, active treatment, reducing treatment, maintenance phase on minimal treatment or complete remission off treatment
- Current treatment
The Bullous Pemphigoid Disease Area Index (BPDAI) described by an expert panel  has separate scores for skin and mucous membrane activity.
Blood pressure, body weight, DEXA bone scan, and blood tests are required to monitor therapy, as medications used for bullous pemphigoid may have serious side effects in some patients.
- Definitions and outcome measures for bullous pemphigoid: Recommendations by an international panel of experts. Journal of the American Academy of Dermatology, Volume 66, Issue 3, March 2012, Pages 479-485. Dedee F. Murrell, Benjamin S. Daniel, Pascal Joly, Luca Borradori, Masayuki Amagai, Takashi Hashimoto, Frédéric Caux, Branka Marinovic, Animesh A. Sinha, Michael Hertl, Philippe Bernard, David Sirois, Giuseppe Cianchini, Janet A. Fairley, Marcel F. Jonkman, Amit G. Pandya, David Rubenstein, Detlef Zillikens, Aimee S. Payne, David Woodley, et al.
- British Association of Dermatologists’ guidelines for the management of bullous pemphigoid 2012. VA Venning, K Taghipour, MF Mohd Mustapa, AS Highet and G Kirtschig, BJD, Vol. 167, No. 6, December 2012 (p1200-1214)
- Chen YJ, Wu CY, Lin MW, Chen TJ, Liao KK, Chen YC, Hwang CY, Chu SY, Chen CC, Lee DD, Chang YT, Wang WJ, Liu HN. Comorbidity profiles among patients with bullous pemphigoid: a nationwide population-based study. Br J Dermatol. 2011 Sep;165(3):593-9. doi: 10.1111/j.1365-2133.2011.10386.x. Epub 2011 Jul 28.
On DermNet NZ:
- Bullous pemphigoid – pathology
- Childhood bullous pemphigoid
- Blistering skin diseases
- Cicatricial pemphigoid
- Epidermolysis bullosa acquisita
- Oral blistering diseases
- International Pemphigus & Pemphigoid Foundation
- Bullous pemphigoid – Medscape Reference
- Pemphigoid – British Association of Dermatologists
- Australasian Blistering Diseases Foundation
- Patient information: Bullous pemphigoid (The Basics) – UpToDate (for subscribers)
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