Bullous pemphigoid is an autoimmune disease of the skin that is associated with blistering and whose incidence increases significantly after the age of 60. With approximately 0.7 to 1.8 new cases per 100 000 population annually, bullous pemphigoid is a rare disease, although it is considered the most common blistering autoimmune dermatosis.
What is bullous pemphigoid?
Bullous pemphigoid is an autoimmune disease of the skin (autoimmune dermatosis) associated with subepidermal, turgid blisters (bullae). The not infrequently hemorrhagic (leading to bleeding) blisters can manifest themselves on reddened (on erythema) and healthy skin. In particular, the abdomen and the intertrigines (including the axillae, flexor sides of the extremities, inguinal region, gluteal fold) are affected by blister formation, while the oral mucosa is generally not involved (in about 20 percent). Bullous pemphigoid is often associated with certain autoimmune diseases such as ulcerative colitis, polymyositis, or chronic polyarthritis, and in rare cases with malignant tumors. In addition to the bulging, firm blisters that are considered the leading symptom of bullous pemphigoid and may be heralded in some cases by itching or wheals (urticaria), pyoderma (purulent inflammation of the skin) may develop as a result of secondary streptococcal or staphylococcal infection.
Causes
Bullous pemphigoid represents an autoimmune disease and is accordingly due to a dysregulation of the immune system. In this case, the immune system produces the body’s own autoantibodies, so-called immunoglobulins G (IgG), which are directed against certain proteins of the hemidesmosomes, cellular components of the cell membrane that ensure the connection between the epidermis and basement membrane (skin layer lying between the epidermis and dermis or dermis). Macrophages (scavenger cells) and monocytes (precursor of macrophages) destroy the areas of hemidesmosomes erroneously marked as pernicious (malignant) by the autoantibodies, so that adhesion (cohesion) between the affected skin layers is no longer ensured. This leads to fluid retention and the formation of blisters. Exactly what causes this dysregulation is not clearly understood. It is known that bullous pemphigoid can be triggered by drugs such as furosemide, diazepam, diuretics, or ACE inhibitors, certain malignant tumors (including bronchial, prostate carcinoma), and UV radiation.
Symptoms, complaints, and signs
Bullous pemphigoid is primarily manifested by the noticeable skin changes. Most people develop redness and reddened nodules, some of which are swollen and intensely itchy. After weeks or months, small blisters develop from these elevations. These may appear on the reddened skin, but often spread to healthy areas of skin as well. They are usually a few millimeters to two centimeters in size and filled with a clear, yellowish fluid. Occasionally, the blisters are partially filled with blood. The cover of the blisters is formed by the epidermis, which is why a bullous pemphigoid is usually very resistant and bulging. If it opens, superficial, slightly bleeding and weeping skin defects usually appear at the affected site. Often, redness, swelling, nodules and skin defects coexist and form the skin appearance characteristic of the disease. The skin changes can occur on the entire body. They form particularly frequently on the abdomen, armpits, groin, inner thighs and flexor sides of the arms. In one in five patients, the oral mucosa or conjunctiva are affected. The painful erosions occur in episodes and often heal on their own. In the absence of treatment, bullous pemphigoid can persist for years.
Diagnosis and course
Bullous pemphigoid can usually (about 80 to 90 percent) be diagnosed, in addition to the leading clinical symptom (bulging blisters), on the basis of the detection of autoantibodies (IgG and its complement C3) to hemidesmosomes in the lamina lucida of the basement membrane (direct immunofluorescence) or in serum (indirect immunofluorescence) by fluorescent anti-antibodies. In addition, in some cases the erythrocyte sedimentation rate is increased and peripheral eosinophilia (increased concentration of eosinophilic granulocytes) or other immunoglobulins (IgE) are detectable in the serum.Histologically (fine tissue), infiltrates of the dermis by eosinophilic and neutrophilic granulocytes (certain leukocytes) as well as lymphocytes and histiocytes can be detected. Imaging techniques (chest x-ray, abdominal sonography) and a blood stain can be used to rule out tumors as triggers. In most cases, bullous pemphigoid develops spontaneously and has a recurrent, relapsing course. Untreated, bullous pemphigoid is lethal (fatal) in 30 to 40 percent of cases.
Complications
As a rule, bullous pemphigoid significantly reduces the patient’s quality of life. There is severe and very unpleasant discomfort on the skin of the affected person. Itching develops, which is associated with redness of the skin. If the patient scratches the skin, the itching usually intensifies. Due to the occurring loss of appetite, there is also a strong loss of weight and thus often underweight. As a result, the patient feels weakened. High fever also occurs. The treatment itself is carried out with the help of antibiotics and immunosuppressants. These can lead to minor side effects, but these depend on the patient’s health condition. In most cases, taking the medicine results in a positive course of the disease. However, the affected person may develop the disease again later in life. If treatment with medication is not successful, immunoglobulins can be administered intravenously. As a rule, this leads to a positive course of the disease. Further complications do not occur. The patient’s life expectancy is also not altered.
When should you see a doctor?
If itching, redness of the skin, or the characteristic skin blisters are noticed, a physician should be informed. Especially if symptoms increase, it is best to consult a medical professional quickly. This is especially true if the symptoms significantly affect the well-being. It is advisable to have a possible bullous pemphigoid clarified quickly before further complications arise. Anyone who notices physical exhaustion and sometimes isolation from social contacts in a relative should address this immediately. With support, the visit to the doctor and the subsequent treatment are often easier. The skin disease occurs predominantly in older people – in addition to the recommended routine examinations, a doctor should be consulted immediately in the event of unusual symptoms. If bullous pemphigoid is actually diagnosed, it can usually be treated well. Since drug therapy carries certain risks, the following applies: consult your doctor regularly. In case of severe side effects, an appointment should be made with the family doctor. Other contacts are the dermatologist or – in case of severe symptoms – the medical emergency service.
Treatment and therapy
In bullous pemphigoid, therapy consists of topical or local as well as systemic treatment measures and is aimed at immunosuppression by reducing autoantibody synthesis with medication using immunosuppressants or steroids. The therapeutic measures depend on the extent of the disease, the age and existing underlying diseases of the specific affected person as well as on the extent (localized or generalized) and the trigger of the skin disease. If the pemphigoid is drug-induced, for example, the triggering substances must be discontinued or changed accordingly. In the case of a mild bullous pemphigoid, an effective topical glucocorticoid or steroid is sufficient for treatment in many cases. This is applied to the affected skin areas in combination with antiseptic ointments or creams such as clioquinol cream, ethacridine lactate ointment, and cadexomer iodine. In all cases, blisters are opened and punctured prior to topical treatment. For systemic or internal treatment, oral or intravenously infused glucocorticoids are additionally used in bullous pemhigoids of moderate severity, which are combined with azathioprine and the dose of which is successively (gradually) reduced in the course of therapy. The oral use of niacinamide (nicotinamide) and tetracycline in combination with topical glucocorticoids is considered equally successful, although such therapy acts on the local inflammatory mediators rather than inhibiting antibody formation.In isolated cases, high-dose corticosteroids, intravenously infused immunoglobulins, or plasmapheresis are used, which is generally not required in the case of common bullous pemphigoid.
Outlook and prognosis
In this disease, most patients are dependent on lifelong therapy because the disease cannot be treated causally. If the disease occurs as a result of various triggering substances, these must be discontinued to relieve symptoms. Patients usually depend on taking medication and using various creams and ointments to improve the quality of life and combat the symptoms. The exact treatment is also highly dependent on the severity of the symptoms. Particular complications do not occur during treatment and there is relief of symptoms. If the disease is not treated, the symptoms remain and have a very negative effect on the patient’s quality of life and everyday life. This results not only in restrictions in everyday life, but often also in psychological complaints or the development of depression. For this reason, treatment is necessary in any case. The life expectancy of the patient is not limited by the disease. In some cases, the symptoms disappear at an older age, although this course has not yet been fully clarified.
Prevention
Because the exact triggers for manifestation of bullous pemphigoid are not conclusively understood, it cannot be directly prevented. Known potential factors that may promote pemphigoid development (including medications) should be avoided, if appropriate.
Follow-up
In most cases, no specific or direct measures or options for aftercare are available to the affected person in this case. The affected person is therefore primarily dependent on a rapid and, above all, early diagnosis and treatment of this disease, so that further complications can be prevented. It is also not possible for the disease to heal itself, so that treatment is absolutely necessary. The treatment itself is usually carried out with the help of medication. These should be taken regularly and in the correct dosage to prevent further complications. A doctor should always be contacted first if there are any questions or if anything is unclear, so that no further complaints arise. Unfortunately, most of those affected by this disease are also dependent on dialysis. They often need the support and care of friends and family. Sensitive and caring conversations are also necessary so that psychological upsets or depression do not occur. It cannot be universally predicted whether the disease will lead to a reduction in life expectancy. Further measures of an aftercare are not possible in this case.
What you can do yourself
Bullous pemphigoid is a rare autoimmune disease of the skin that primarily affects individuals over the age of 60. The causes have not yet been conclusively determined, but a connection between the disease and certain medications, for example diuretics or ACE inhibitors, is suspected. If patients notice symptoms of the disease, they should definitely consult a doctor or specialist clinic specializing in autoimmune diseases as soon as possible and inform their contacts about the medications they are taking. Patients usually suffer from very severe itching, which is usually treated by a doctor. On vacation or in other exceptional situations, antihistamines, which are available over-the-counter as ointments, tablets or drops in pharmacies, also help. The itching often increases with heat. Many patients suffer from particularly severe itching under the covers at night. Here it can help to exchange conventional comforters for light polar fleece blankets. These textiles are very light and breathable, so that heat build-up does not occur. If blisters occur on the body, care should be taken to wear loose-fitting clothing and underwear with a high percentage of breathable materials such as cotton or bamboo. If blisters occur on areas of the body that cannot be covered with clothing, these skin changes often have a disfiguring effect. With the help of special make-up from the pharmacy or cosmetics store, the blisters can be visually concealed.
