Bullous pemphigoid - Causes, Symptoms, Treatment ... - Health Inset

What is Bullous pemphigoid?

Bullous pemphigoid is a dermatological condition represented by blistering eruptions on the surface of the skin that are normally called bullae. According to the International Pemphigus and Pemphigoid Foundation, this is a type of an autoimmune skin disorder that manifests in very few individuals.

Bullous pemphigoid Incidence

The disorder is rarely spotted in children and inflicts individuals above the age of 70. In the UK, this condition is estimated at 4.28 per 100,000 person years. On the other hand, in France it is in the order of 0.6-0.7 cases per 100,000 persons per year. Both males and females are equally affected by the ailment.

Bullous pemphigoid Symptoms

In this condition, the skin (that appears normal otherwise) gets filled with crops of tense elevated lesions. These blisters are usually filled with clear, cloudy or blood-stained fluid. The initial eruptions on the cutaneous region are generally hives- a type of skin rash with pale red, raised and itchy bumps. ?Blisters develop only after a week or more. In some cases, they do not appear until months later. At this stage, the degree of itch can vary from severe to mild. The burning sensation of the skin makes the condition even more distressful. The skin around the blisters may however, remain normal without any pruritic sensation.

Picture 1 -?Bullous pemphigoid

The blistering eruptions can occur anywhere on the body, but are significantly present on the abdomen, groin, trunk, upper arms, armpits and inner thighs. The severity of the blistering can vary. In some cases, they may affect only a specific area of the body such as the lower leg or arm. These rounded protrusions find it easier to develop along creases or folds in the skin. In rare occasions, the mucosal blisters affect the inner lining tissue of the mouth. Oral blisters can cause burning pain and make eating difficult, owing to sensitivity to acidic food.

When the deeper regions of the throat undergo blistering, swallowing gives rise to cough. The pus-filled lumps can sometimes break to form mouth ulcers. The oral sores can even affect the spongy gums and cause persistent bleeding. Painful sores that form on the inside of the nose can predispose patients to nosebleeds.

Bullous pemphigoid Causes

The condition is believed to be a type 2 hypersensitivity reaction, in which the antibodies called immunoglobins produced by the immune system bind to antigens on the own cell surfaces of a patient. These antigens are basically proteins called hemidesmosomal antigens in the basement membrane ? a thin membrane composed of a single layer of cells present between the epidermis and dermis of the skin. The inflammatory response produced in this autoimmune skin disorder, owing to the autoantibody attack, causes accumulation of fluid in the basement membrane. In the course of time blisters begin to erupt on the skin, causing acute inflammation. There is no clear explanation given about the cause of the autoimmune reaction in the body. The following factors may trigger the formation of blisters:

• Radiation treatment
• Exposure to ultraviolet light
• Prescribed drugs such as NSAIDs, furosemide, captopril, ibuprofen, penicillamine and antibiotics
• Disorders like Parkinson disease, multiple sclerosis, ulcerative colitis, stroke and dementia
• Genetic origin
• Post-vaccination side effects

Bullous pemphigoid Diagnosis

Physical inspection of the skin by a well-experienced dermatologist is important as a part of primary diagnosis. However, a plain observation does not reveal much about the underlying cause. The primary symptoms of the condition closely resemble a simple skin rash such as eczema. Therefore, a biopsy of the affected skin should be done to confirm that these blisters are a consequence of an autoimmune condition. A series of biopsy samples are collected and sent for some further tests like:

Immunofluorescence

Fluorescein-stained or labeled antibodies are used in this procedure to locate antigens in a sample of cells. These antibodies recognize the target proteins in the basement membrane and binds to them. A fluorescence microscope is then used to visualize the distribution of these target molecules through the biopsy sample.

Hematoxylin and eosin (H&E) stain

The staining technique involves application of hemalum, which is a solution of hematoxylin and alum used to color the nuclei of the cells in the skin sample. It is followed by counterstaining of the cells with eosin, a fluorescent dye that normally stains only esonophilic structures composed of proteins. The differential staining method helps in distinguishing the red-stained antibodies from the remaining structures.

An analysis on a sample of a patient?s blood or urine or both can confirm the presence of antibodies responsible for causing the condition. The blister fluid can also be tested in a pathological laboratory for closer examination of the basement membrane.

Bullous pemphigoid Differential Diagnosis

The differential diagnosis of the disease aims at distinguishing it from other skin blistering ailments like:

• Linear IgA dermatosis
• Drug-Induced Bullous Disorders
• Epidermolysis bullosa
• Erythema multiforme
• Dermatitis herpetiformis
• Cicatricial pemphigoid
• Systemic Lupus Erythematosus

Bullous pemphigoid Treatment

In mild cases, the lesions normally heal without scarring the skin. A rough patch of skin is left when a blister bursts, which eventually heals. In other cases, the blister fluid is absorbed into the body and the roof of the blister settles back down on the skin. The symptoms can be resolved using high-strength steroid creams. Oral administration of high dose of cortisone ensures faster symptomatic relief. Prednisolone is a common steroid tablet prescribed to a number of patients. However, some medical experts suggest that topical steroids are more effective than tablets and have lesser side-effects. Systemic steroids along with immunosuppressant drugs like azathioprine, mycophenolate mofetil, and methotrexate are needed to control the prolonged episodes of itching and blistering. The severity of the condition determines the exact dose of the combination medication.

Picture 2 -?Bullous pemphigoid Image

Intravenous immunoglobins infusions could be given on a monthly basis in extensive blister formations. Patients who are allergic to steroids can opt for antibiotics such as erythromycin, dapsone, sulphonamide and tetracycline to prevent the spread of the disorder. The raw areas of the skin may require therapeutic measures like dressings and wound management. An intravenous drug called rituximab is prescribed to individuals who are unresponsive to other types of therapy.

Bullous pemphigoid Complications

After the blisters have bursted, the raw areas of the skin are sometimes prone to a host of secondary infections that could be detrimental to the overall health of the patients. The infection can also spread to the lungs, causing pneumonia. Mouth sores can contribute to a decrease in appetite.

Bullous pemphigoid Prognosis

In the majority of patients, the condition is self-limited with the blisters resolving within five years. With treatment, the skin sores remit within 1.5 to 5 years. The medications can be discontinued after this period. Approximately 6-41% of elderly patients with the condition have a fatal outcome. However, the availability of various treatment options in the USA has significantly brought down the mortality rate.

The management of Bullous pemphigoid requires close monitoring and regular use of the prescribed drugs. Seek immediate medical advice if the blisters have occupied a large part of the body. Timely cure can prevent the development of skin infection.

References:

http://dermnetnz.org/immune/pemphigoid.html

http://www.mayoclinic.com/health/bullous-pemphigoid/DS00722

http://en.wikipedia.org/wiki/Bullous_pemphigoid

http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001886/

Source: http://www.healthinset.com/bullous-pemphigoid.html

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