O r a l M e d i c i n eCase Reports

Gingival involvementin mucous membrane pemphigoid

R Y. Bozkurt, DDS, PhDVH. Çeienligil, DDS, PhD**/A. Sungur, MD**VÇ. Ruacan, MD*

A 60-year-old woman with clinical features of desquamative gingivitis had a history of painful, blisteringgingival lesions for more than 2 years. There were no Other accompanying tnucosal or skin lesions.Clinical examination revealed erythematous and edeniatous gingiva with ulcerated areas and evidenceof intact and ruptured bullae. White plaquelike lesions were also noted. Gingival manipulation causedepitheliai desquwnation. Light microscopic examination of biopsy specimens from the perile.sional gingi-val tissue showed separation of the oral gingival epithelium and connective tissue at the margin of thecollapsed bulla. A moderately intense inflammatory infiltrate was present in the connective tissue. Directimmunofluorescent microscopy revealed a continuous linear deposition of immunoglohulin G and C3 atthe basement membrane zone. On the basis of clinical, histopathologic, and immunofluorescent findings,the diagnosis of mucous membrane pemphigoid was maile. (Quintessence Irtt 1998;29:438-441)

Key words: bulla, desquamative gingivitis, direct immunofluorescence, mucous membrane pempbigoid

Desquamative gingivitis is a nonspecific but raretn an if estático of a variety of diseases, rather than a

distinct clinical entity. It is characterized hy erosive,ulcerative. and/or vesiculobullous lesions of the gingivafeaturing epithelial desquamation,' Although systemicfactors such as endocrine imbalances, chronic infections,and drug reactions are considered to play a role in thepathogenesis of desquamative gingivitis,'--' dermatosesare shown as the major factor in 75% of cases.''''

'Assistant Professor, Department of Peri odontology, Faeulty of Den-tistry, Süleyman Demirel University, Isparta, Turkey.

"**Profes.'(or, Depanment of Periodontology, Faculty of Dentistry,Hacettepe University, Ankara, Turkey,

*''''Assoeiate Professor, Department of Pathology, Faculty of Medicine,Hacettepe University, Ankara, Turkey.

**"Professor, Department of Pathology, Faculty of Medicine, HacettepeUniversity, Ankara, Turkey.

Reprint requests; Prof Dr Haviye Çeienligil, Department of Periodon-tology, Faculty of Dentistry, Hacettepe University, OûlOO, Ankara,Turkey. E-niail: hcO4-k@tr-net.net,tr

Mucous membrane petnphigoid (also known as cica-tricial pemphigoid) is a relatively rare chronic vesiculc-bullous disease that affects mucosal surfaces, includingthe conjunctiva, oral cavity, larynx, esophagus, upperrespiratory tract, and genitalia, as well as skin,*'Recurrent lesions result in permanent scarring thatcauses morbidity and mortality.^ Likewise, ocular le-sions can be severe and tnay eventually causeblindness,''

Desquamative gingivitis is the most characteristicfeature of mucous membrane pemphigoid and maymark the onset of the disease,' However, gingival in-volvement in the ahsence of other manife,';tations pre-sents a challenging diagnostic and therapeutic problem.In such cases, tbe identification of the underlying patho-sis of tbe gingival lesions is of utmost importance.

Tbis report describes a case of mucous membranepempbigoid in wbich tbe lesions were confined togingiva. Clinical signs and symptoms, histologie char-acteristics, and direct itnmunofluorescent features arediscussed.

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Fig 1 Edematous gingiva witti a patchy distribuiíon oí whiteareas. Fig 2 Bulla at the right mandibular premolar area and an ulcer

on llie molar site Note the erosion in the interdenial papilia be-tween the maxiilaiy right canine and lirst premolar.

Fig 3 White plaqueiike lesions on the gingiva oí themaxillary left premolars.

Fig 4 Remnant of a ruptured bulla on the gingiva olthe mandibuiar ielt premolars. Note the intact buila onthe retromoiar area.

CASE REPORT

A 60-year-old woman was referred to the Department ofPeriodontology of Hacettepe University by her physi-cian. She had a 2-year history of burning scn.̂ ations andpainful, hlisteririg gingival lesions. No other mucosal orskin lesions had been present at any time. Her medicalhistory was unremarkable.

Intraoral examination revealed erythematous andedematous gingiva with a patchy distribution of whiteareas (Fig 1), The gingival epithelium could easily bepeeled from the underlying connective tissue, A buHawas observed at the mandibular right premolar area andan ulcer was present at the molar site (Figs 1 and 2), Anerosive lesion was observed in the interdental papillabetween the maxillary right canine and premolar (Fig2), White plaqueiike lesions were present on the gin-giva of the maxillary left premolars (Fig 3), A rupturedbulla was noted on the lingual aspect of the gingiva inthe mandibular left premolar area (Fig 4), There wasalso an intaci bulla on ihe left retromoiar area.Examination of the palate revealed large ulceratedareas, indicating previous bullous formations. Scarringcaused by tbe recurrent lesions was also noted on thepalatal mucosa (Fig 5),

Fig 5 Large ulcerated areas, indicatrng previous buiious forma-tions Scarring, secondary tc the blistering, is aisc present.

Two biopsy specimens were obtained from the peri-lesional gingival tissue. Light micrcscopic examinationof the sections, slained with hematoxylin and eosin,showed .separation of the intact oral gingival epitheliumfrom the underlying connective tissue with subepithelialbullous formation (Fig 6), Acatithosis and hyperkerato-sis of the epithelium were also noted. In the upper partsof the stroma, subjacent to the cleavage sites, a moder-

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Fig 6 (Left) Intact epithelium separated from the underiying con-nective tissue with subepithelial bulious formation. (Hematoxyiinand eosin; original magnifioation x230.)

Fig 7 (Below) Linear immunogiobuiin G deposition at the base-ment membrane. (Direct immunofluorescence; originai magnifica-tion xi 15.)

Ikr '.'

ate infiltration of chronic inflammatory cells was ob-served (Fig 6). Direct immunofluore.scent studies re-vealed a linear deposition of immunoglobulin G and C3at the basement membrane ^one (Fig 7). No evidence ofintercellular staining was noted. An indirect immunoflu-orescence test was not performed.

On the basis of clinical, histopathologic, and directimmunofluorescent findings, the diagnosis of mucousmetnbrane pempbigoid was made. The patient was thenreferred to a dermatologist. She was treated with 60 mgdehydrocorticosterone daily for 15 days. Meanwhile,the patient was instructed in plaque control and told touse a soft toothhrush and peroxide mouthwash two orthree times a day. Scaling and root planing were alsoperformed in the clinic.

The lesion.s improved considerably. Then, topicalsteroid (triamcinolone, 0.1% ointment) treatment wasgiven for 3 weeks. However, the patient did not come tosubseqtient recall visits.

DISCUSSION

Mucous membrane pemphigoid is an unusual chronicvesicuiobullous disease that primarily involves mucousmembranes. Oral, ocular, and genital mucous mem-branes are most frequently affected."'' The onset of thedisease is more common between the fourth and eighth

decades of life,* although it has been documented inchildren and adole.scents.'*"' Females are affected muchmore frequently Chan males.''

The most characteristic feature of oral involvement isan erosive or desquamative gingivitis with areas ofdesquamation, ulcération, and vesiculation of the at-tached gingiva." Nisengard and Rogers" analyzed 174cases of desquamative gingivitis with the use of directimmunofluorescence and showed that approximately50% of the cases were mucous membrane pemphigoid.

The clinical presentation in this patient was highlysuggestive of mucous membrane pempbigoid in a fe-male patient at the age of 60 years, but the lesions wereconfined exclusively to gingiva and there were no ac-companying ocular or other mucosal lesions. Therefore,other vesiculobniious diseases, sucb as bullous pem-phigoid, pemphigus, erythema multiforme, and bullouslichen planus, were considered in the differential diag-nosis." Microscopic examination of the biopsy speci-mens revealed subepitbelial bullous fonnation with anintact basal cell layer and chronic inflammatory infil-trate. Direct immunofluorescence studies revealed de-position of immnnoglobulin G and C3 at the basemetitmembrane zone.

Mucous membrane pemphigoid and bullons pem-phigoid presetit similar histopathologic and immuno-pathologic features. The presence of scarring secondary

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to the blistering is considered to be a major diagnostiefeature to distinguish the diseases, Furtbermore, mucousmembrane pemphigoid mainly affects mucosal tissues,whereas skin is the primary tissue involved in bullouspemphigoid.'̂ Gallagher and Shklar^ stated that the onlyfeature that distinguishes mucous memhrane pem-phigoid from bullous pemphigoid is "extensive skin in-volvement with large bullous lesions," The patient inthe present case had features typieal of mucous mem-brane pemphigoid, including the presence of scarringlesions and the laek of skin involvement.

Desquamative gingivitis may also be present in pem-phigus as a rare manifestation, and the vesicular iesionsmay resemble those of mucous membrane pemphigoid.In this patient, light and direct immunofluorescentmicroscopic examination of the biopsy specimens ruledout pemphigus by revealing the absence of acantholyticchanges and the presence of intercellular immunoglohu-lin G deposition in oral gingival epithelium.'''

Erythema multiforme is an inflammatory disease in-volving the skin and oral mucosa. Although extensivebullous and ulcerative lesions may develop in this con-dition, desquamative gingivitis is not seen.'̂ Moreover,the onset of the disease is usually acute, unlike mucousmemhrane pemphigoid. Large labial lesions are themost characteristic feature of erythema multiforme.'''Microscopic analysis of an oral lesion also reveals anunusual degeneration of the upper stratum spinosum ofthe oral epithelium, which is specific for this entity.'^

Bullous-type oral lichen planus may also resemblemucotis memhrane pemphigoid. However, the charac-teristic white striations of liehen planus are also evidentin the bullous type.'-' Furthennore, microscopic evalua-tion of the oral lesions reveals hydropic degeneration ofthe basal layer of the oral epithelium.'*

Management of mucous membrane pemphigoid re-quires hoth accurate diagnosis and treatment. This pa-tient was prescribed systemie corticosteroid therapy bythe dermatologist, Periodontal management is also con-sidered an essential part in the overall treatment becausethe gingival involvement may represent an exaggeratedresponse to bacterial plaque.'•" Therefore, the patientwas motivated for plaque control, and initial phases ofperiodontal treatment were performed to eliminate thelocal irritants. The lesions improved considerably; how-ever, because the patient did not eome to the recall vis-its, follow-up evaluation of the lesions could not beachieved.

The diagnosis of a potentially damaging bullous dis-ease, made in light of the gingival features, confirms theimponance of histopathologic and immunohistologicexamination in identifying the underlying cause ofdesquamative gingivitis.

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