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Pemphigus Vulgaris: Health Significance As A Skin Disease, Diagnosis, Prognosis And Treatment

Updated on February 20, 2014

Clinical Presentation Of Pemphigus Vulgaris

Eruptions may be present continuously over long periods or may appear intermittently in pemphigus vulgaris. Pressure on the bullae enlarge them (Nickolsky’s sign). This is because of splitting of the skin due to acantholysis. The bullae contain clear
Eruptions may be present continuously over long periods or may appear intermittently in pemphigus vulgaris. Pressure on the bullae enlarge them (Nickolsky’s sign). This is because of splitting of the skin due to acantholysis. The bullae contain clear | Source

A Clinical Overview

Eruptions may be present continuously over long periods or may appear intermittently in pemphigus vulgaris. Pressure on the bullae enlarge them (Nickolsky’s sign). This is because of splitting of the skin due to acantholysis. The bullae contain clear serous fluid or purulent or hemorrhagic material. When they rupture, the lesions become crusted.

Healing results in pigmentation and scarring. Mucosal lesions occur in the mouth, nose, conjunctiva and vagina. The oral lesions may extend to the pharynx and larynx to cause dysphagia or hoarseness of voice.

Mild fever may occur. Hypoproteinemia results from extensive denudation and oozing, hypochromic anemia and hyponatermia may develop also. Blood examination reveals leukocytosis and raise ESR levels. Serious complications include secondary impetigo, ulceration and gangrene, gastrointestinal upsets and pneumonia.

Diagnosis: Pemphigus has to be diagnosed clinically and confirmed by histological examination. Pemphigus vulgaris has to be distinguished from dermatitis herpetiformis, other bullous lesions and drug eruptions as they are quite similar to each other and an inexperienced health worker can mistake it for others or mistake others for it.

Differentiating Features Of Bullous Dermatitis Herpetiformis And Pemphigus Vulgaris

 
Dermatitis herpetiformis
Pemphigus Vulgaris
Age of onset
20 to 40 years
25 to 50 years or above
Pruritis
Severe
mild or nil
Distribution of bullae
Symmetrical skin, lesions on both sides of forearm, scapular regions, and buttocks affected the most. Mucous membranes are usually not affected.
Asymmetrical lesions over face, neck and trunk. Mucous membranes are usually affected
Erythema
Present
Not present
Nikolsky’s sign
Negative
Positive
General health
Unaffected
Deteriorates gradually
Prognosis
Chronic course, does not shorten life
Untreated, prognosis is bad
Drug Therapy
Dapson and Sulpha drugs
Corticosteroids

Prognosis

Prognosis: Untreated cases follow a chronic recurrent course marked by remissions and exacerbations, but ultimately ending fatally. Corticosteroids have improved the prognosis for life and health considerably.

Treatment Of Pemphigus Vulgaris

Untreated cases follow a chronic recurrent course marked by remissions and exacerbations, but ultimately ending fatally. Corticosteroids have improved the prognosis for life and health considerably.
Untreated cases follow a chronic recurrent course marked by remissions and exacerbations, but ultimately ending fatally. Corticosteroids have improved the prognosis for life and health considerably. | Source

Treatment

Specific drugs are not available.

General management: Proper nursing and nourishing diet are important. If secondary infection is evident, broad-spectrum antibiotics are indicated.

Corticosteroids: These bring down the skin lesions and general symptoms rapidly. Buccal lesions are not so readily responsive. Prednisolone in a daily dose of 40 to 60 mg or its equivalent of the newer derivatives should be started. The dosage can be reduced as the condition improves. Maintenance treatment may be required for prolonged periods to prevent relapse. Cyclophosphamide and azathioprine may be tried as immunosuppressant drugs in selected cases.

Local Treatment: This consists of the application of gentian violet and as the lesions tend to heal, soothing creams. If oral lesions occur, mouth washes with saline and local application of gentian violet or boroglycerine give relief.

Other forms of pemphigus: These include pemphigus foliaceous, benign pemphigus of mucous membranes and familial benign chronic pemphigus. These are all rare compared to pemphigus vulgaris.

© 2014 Funom Theophilus Makama

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