Pathology of the Head and Neck

74 J.W. Eveson3.2.4 Herpangina3Herpangina is also caused by a variety of group A coxsackieviruses including A1 to 6, 8, 10, and 22. Othercauses include coxsackie group B (strains 1–4), echoviruses,and other enteroviruses [161]. It is highly contagiousand tends to affect young children in the summerand early autumn period. Like hand-foot-and-mouthdisease, it rapidly spreads through close-knit communities,such as schools, and presents with acute pharyngitis,anorexia and dysphagia, with or without cervicallymphadenopathy. Typically, the lesions are restricted tothe soft palate, uvula, anterior pillars of the fauces andpalatine tonsils. They consist of multiple, small vesiclesthat rapidly rupture to form superficial ulcers, whichmay coalesce. In addition, there is often more generalisedoropharyngeal erythema. The condition usuallylasts 1–2 weeks and is treated symptomatically.3.2.5 Pemphigus VulgarisFig. 3.2. Pemphigus vulgaris showing suprabasal clefting and acantholyticcells in an intraepithelial blisterPemphigus vulgaris is an uncommon, but potentiallylethal, mucocutaneous disorder that occasionally alsoinvolves the eyes. It is an autoimmune disease due to circulatingantibodies against the intercellular attachmentsof stratified squamous epithelia [163]. The specific targetappears to be desmoglein 3 [2]. However, 50% of patientswith pemphigus vulgaris also have autoantibodiesto Dsg1, but cases that are predominantly oral have onlyDsg3 antibodies [70]. Associations have been describedbetween pemphigus vulgaris, myasthenia gravis andthymoma and a variety of drugs, including penicillamine,rifampicin and captopril. In addition, some casesare associated with internal malignancies, particularlyof the haematolymphoid system, and the condition isthen termed paraneoplastic pemphigus.Pemphigus is more common in Asians and AshkenaziJews than other races and most patients are in the fourthor fifth decades. The mouth is the most common site ofinitial involvement and remains the only site affected inabout half of patients. The oral features are very variable.It is uncommon to find intact vesicles and most patientspresent with painful, ragged superficial ulcers and areasof boggy and shredded mucosa. The buccal mucosa, gingivaand soft palate are the most common sites. In thetongue the condition may present as deep, non-healingfissures. Fluid from intact or recently ruptured blistersmay contain acantholytic ( Tzanck) cells, although thisis rarely used as a diagnostic measure. The disease maybe relatively mild or even regress, but some cases, particularlythose with extensive cutaneous involvement, maybe fulminant, either as a consequence of the disease itself,or as a complication of medical treatment.Microscopy shows suprabasal clefting of the epitheliumdue to loss of intercellular attachments and acantholysis(Fig 3.2). A single layer of keratinocytes may remainattached to the corium by their hemidesmosomes,but the cells are separated from each other laterally toform a characteristic “tombstone” appearance. The acantholyticcells floating in the vesicular fluid are rounded,with condensed cytoplasm surrounding hyperchromaticnuclei. The vesicles may contain acute and chronicinflammatory cells, and eosinophils may be a conspicuousfeature. In many cases, the roof of the blister is lostduring the biopsy procedure due to a positive Nikolskyphenomenon, but a row of keratinocytes remains adherentto the floor. Direct immunofluorescence on frozentissue shows deposits of IgG and less frequently IgM andIgA in the intercellular junctions, producing a characteristic“chicken wire” appearance.3.2.6 Pemphigus VegetansPemphigus vegetans is considerably less common in themouth than pemphigus vulgaris [15]. It usually presentsclinically as serpiginous ulcers that are most frequent onthe dorsum of the tongue and lips [187]. The lingual lesionsclosely resemble those of erythema migrans. Thepapillomatous, proliferative lesions that characterisecutaneous pemphigus vegetans can sometimes be seenat the angles of the mouth. As in pemphigus vulgaris,drugs, particularly ACE inhibitors, have been invokedas possible causative agents in some cases [12, 137].Microscopically, the epithelium tends to proliferateand become verruciform. Acantholytic cells may not beconspicuous and eosinophil microabscesses are said tobe the most typical histological feature. However, thelesions frequently resemble pyostomatitis vegetans andconventional microscopy may not be diagnostic. Thepresence of typical skin lesions often helps in makingthe diagnosis and it may be differentiated from pyostomatitisby the clinical picture and appropriate immunocytochemicalinvestigations [73].