Pemphigus vulgaris is a potentially fatal blistering disease occurring in all races but commoner in Ashkenazi Jews and possibly in people from the Indian subcontinent. Onset is usually in middle age and both sexes are affected equally. Pemphigus is derived from the Greek word pemphix meaning bubble or blister. Pemphigus describes a group of chronic bullous diseases, originally named by Wichman in 1791.
Prior to the development of oral steroids this condition was frequently fatal. The development of autoantibodies against the desmosomal protein, desmoglein 3, is pathogenic in this disease and they can be measured experimentally as markers of disease activity. Rarely the disease can be drug induced (e.g. penicillamine or ACE inhibitors).
Skin biopsy shows a superficial intraepidermal split just above the basal layer with acantholysis (separation of individual cells). In the rarer variant, pemphigus foliaceous (characterized by anti-desmoglein 1 autoantibodies), the split is higher in the upper epidermis. Both direct IMF of skin (perilesional) and indirect IMF using patients' serum show intercellular staining of IgG within the epidermis.
Medications that may cause this condition include:
Pemphigus is uncommon. It almost always occurs in middle-aged or older people. There are three types of pemphigus which vary in severity: pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic pemphigus. Note that Hailey-Hailey disease, also called familial benign pemphigus, is an inherited (genetic) skin disease, not an autoimmune disease.
Mucosal involvement (especially oral ulceration) is common and may be the presenting sign in up to 50% of cases. This may then be followed by the appearance of flaccid blisters, particularly involving the trunk. They tend to be sore rather than itchy. Blistering usually becomes widespread but the blisters rapidly denude; thus pemphigus often presents with erythematous, weeping erosions. Blisters can be extended with gentle sliding pressure (Nikolsky's sign). Flexural lesions often have a vegetative appearance. In pemphigus foliaceous the blisters and erosions often start in a seborrhoeic distribution (scalp, face and upper chest) before becoming more widespread.
The skin sores may be described as:
The bullae of Pemphigus vulgaris are intra-epidermal and irregular in shape with acute lateral margins. They are formed by the separation of acantholytic epidermal cells (Tzanck cells). Acantholytic cells may be in the bullae cavity. Dermis beneath the bulla shows number of inflammatory cells including a few lymphocytes and plasma cells.
The skin separates easily when the surface of unaffected skin is rubbed sideways with a cotton swab or finger. This is called a positive Nikolsky's sign.
A skin biopsy is usually done to confirm the diagnosis. These antibodies appear as IgG deposits along the desmosomes between epidermal cells, a pattern reminiscent of chicken wire.
Pemphigus vulgaris is easy to confuse with impetigo and candidiasis.
A blood test for antibodies. This uses methods called immunofluorescence or ELISA to measure the level of the antibody that causes PV (the PV auto-antibody) in your bloodstream.
This is with very high-dose oral prednisolone (60-100 mg daily) or pulsed methylprednisolone and this may need to be lifelong. Therefore other immunosuppressants such as azathioprine (or occasionally cyclophosphamide or ciclosporin) are used as steroid-sparing agents. Intravenous immunoglobulin infusions can be useful in resistant cases.
Whilst treatment is normally effective, perhaps up to 10% of patients may succumb, either because of complications of the disease or more commonly from side-effects of the treatment. It is a difficult disease to control. Intralesional steroids has recently been found to be much more effect than systemic treatment.
Use of azathioprine . Azathioprine can cause bone marrow suppression and an allergic hepatitis. Therefore blood count and liver biochemical tests should be regularly monitored during therapy (every 6 weeks). Pregnancy should be avoided. Long-term use with other immunosuppressants causes a slightly increased risk of malignancy, especially of the skin.
With treatment, lesions can heal normally without scarring. Most patients treated for pemphigus will enter a partial or full remission within 2–5 years.
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