Diagnosis: pemphigus vulgaris
Pemphigus vulgaris is blistering disease involving the skin and mucous membranes, with severe morbidity and occasional mortality. Prior to the development of effective treatment, the disease was 75% fatal within 5 years.1
Its prevalence is equal among men and women, with a rate of occurrence of 0.5/100,000 people per year. The average age of onset is in the fifth and sixth decades of life, but there is wide variation in age. It has multiple causes and risk factors (Table).
The clinical manifestation of pemphigus typically features mucocutaneous blisters followed by erosions. Often they appear first in mucous membranes and may not appear cutaneously until several months later.2 The skin lesions are painful flaccid blisters that may appear anywhere. A characteristic finding of pemphigus vulgaris is the Nikolsky sign, in which lateral stress applied to perilesional skin causes an expansion of the blistering.
There are 2 major subtypes of pemphigus. Pemphigus vulgaris has blisters extending to the deep epidermis, and pemphigus foliaceus has more superficial involvement of the epidermis. Pemphigus can also be seen in paraneoplastics syndromes.
In pemphigus, the epidermal cells lose normal cell contacts and form a blister. Electron microscopy shows desmosomal abnormalities at desmosomal junctions. It is these junctions that guarantee the integrity of the epithelium. Direct immunofluorescence shows IgG deposition in intercellular spaces.
TABLECauses and risk factors for pemphigus vulgaris
| Penicillamine |
| Captopril |
| Rifampin |
| Phenol-based drugs |
| Amide-based drugs |
| Foods: garlic, leek, onion |
| Pregnancy |
| Pesticide exposure |
| Herpes virus infection |
| Cytomegalovirus infection |
| Epstein-Barr virus infectyion |
| Adapted from: Benner et al 2003.4 |
Treatment: Steroids and adjuvant drugs
Inducing remission is the main goal of therapy for pemphigus vulgaris. Epidemiological studies have shown up to a 75% remission rate 10 years after initial diagnosis.3 Corticosteroids are the preferred therapy for the management of pemphigus vulgaris (based on expert opinion). Also, adjuvant drugs such as azathioprine and cyclophosphamide are commonly used in combination with corticosteroids, with the aim of increasing efficacy and of having a steroid-sparing action (level of evidence: 5, expert opinion).