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| Pemphigus Vulgaris |
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Definition: The word pemphigus comes from the Greek pemphix and means bubble or blister. Pemphigus vulgaris (PV) is the most common cause of pemphigus in the United States. Description: It is caused by antibodies directed against desmoglein-3. These antibodies interfere with desmosome function, causing keratinocytes of the suprabasal layer to detach from each other laterally (acantholysis). Keratinocytes remain attached to the underlying dermis because the hemidesmosomes are intact. The end result is the formation of blisters that arise on a normal appearing skin. Blisters are found both on skin and mucous membranes, however in about half of the patients they begin to appear in the mouth. Sera from patients with PV injected intradermally (or intraperitoneally) into neonatal BALB/c mice reproduce the human disease clinically, histologically and immunologically. PV can also be transmitted from mother to newborn. These two facts provide direct evidence that PV is an autoimmune disease mediated by antibodies. It usually presents in the fourth to sixth decades of life, and affects equally males and females. It is extremely rare in pregnancy, and only 27 cases have been reported thus far in the English literature (Ref 7). Similarly to systemic lupus erythematosus, pregnancy precipitates or aggravates the disease, particularly in the first trimester. The presence of PV in pregnancy may affect negatively the product of conception: four of the 27 reported cases of PV in pregnancy ended in intrauterine fetal death . The PV IgG antibodies are transmitted from the mother to the fetus and cause, in about 50% of the cases, skin and mucosal lesions in the neonate, that tend to improve spontaneously within 3 weeks. Histopathology is the most reliable tool of diagnose PV. Like in pemphigus foliaceus, the blisters are in the epidermis, but in PV the acantholysis is deeper. The fluorescent stain highlights the deposition of immunoglobulins along the plasma membrane of keratinocytes in a characteristic fishnet-like pattern. A recent review on the therapy of PV can be in this reference (Ref 8).
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Links to other Resources: www.pemphigus.org |
