Pemphigus Vulgaris

Fig. 3.2. Pemphigus vulgaris showing suprabasal clefting and ac-antholytic cells in an intraepithelial blister

Pemphigus vulgaris is an uncommon, but potentially lethal, mucocutaneous disorder that occasionally also involves the eyes. It is an autoimmune disease due to circulating antibodies against the intercellular attachments of stratified squamous epithelia [163]. The specific target appears to be desmoglein 3 [2]. However, 50% of patients with pemphigus vulgaris also have autoantibodies to Dsg1, but cases that are predominantly oral have only Dsg3 antibodies [70]. Associations have been described between pemphigus vulgaris, myasthenia gravis and thymoma and a variety of drugs, including penicilla-mine, rifampicin and captopril. In addition, some cases are associated with internal malignancies, particularly of the haematolymphoid system, and the condition is then termed paraneoplastic pemphigus.

Pemphigus is more common in Asians and Ashkenazi Jews than other races and most patients are in the fourth or fifth decades. The mouth is the most common site of initial involvement and remains the only site affected in about half of patients. The oral features are very variable. It is uncommon to find intact vesicles and most patients present with painful, ragged superficial ulcers and areas of boggy and shredded mucosa. The buccal mucosa, gingiva and soft palate are the most common sites. In the tongue the condition may present as deep, non-healing fissures. Fluid from intact or recently ruptured blisters may contain acantholytic (Tzanck) cells, although this is rarely used as a diagnostic measure. The disease may be relatively mild or even regress, but some cases, particularly those with extensive cutaneous involvement, may be fulminant, either as a consequence of the disease itself, or as a complication of medical treatment.

Microscopy shows suprabasal clefting of the epithelium due to loss of intercellular attachments and acan-

Fig. 3.2. Pemphigus vulgaris showing suprabasal clefting and ac-antholytic cells in an intraepithelial blister tholysis (Fig 3.2). A single layer of keratinocytes may remain attached to the corium by their hemidesmosomes, but the cells are separated from each other laterally to form a characteristic "tombstone" appearance. The ac-antholytic cells floating in the vesicular fluid are rounded, with condensed cytoplasm surrounding hyperchro-matic nuclei. The vesicles may contain acute and chronic inflammatory cells, and eosinophils may be a conspicuous feature. In many cases, the roof of the blister is lost during the biopsy procedure due to a positive Nikolsky phenomenon, but a row of keratinocytes remains adherent to the floor. Direct immunofluorescence on frozen tissue shows deposits of IgG and less frequently IgM and IgA in the intercellular junctions, producing a characteristic "chicken wire" appearance.

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