pemphigus vulgaris ( pv ) is a chronic autoimmune mucocutaneous disease that usually manifests first in the oral cavity and may later spread to the skin or other mucous membranes . apart from ulcers , vesicles ,
bullae and pustular lesions , it can present solely as mucosal erosions . herein we report a case of intraoral pv presenting as intense erythema and erosions involving the gingiva .
a 52-year - old female presented to the department of periodontics , al - badar dental college and hospital , gulbarga with complaints of bright red gingivae and discomfort in her normal oral function .
the site most severely affected was her upper front buccal gingivae . the considerable pain and discomfort that the patient felt hindered her from carrying out effective oral hygiene measures and this in turn exacerbated the gingival symptoms .
she had essential hypertension , diabetes mellitus and hyperlipidemia and her daily medications included insulin , antihypertensive drugs and statins .
on intraoral examination , there were multiple large irregular erosions and areas of intense erythema involving particularly the gingivae of both upper and lower arch , buccally and palatally / lingually [ figures 1 and 2 ] .
orthopentamogram showed a combination of horizontal and vertical bone loss . the nikolky 's sign ( loss of epithelium occasioned by rubbing apparently unaffected skin ) that is a feature of pv was positive .
the patient had no cutaneous involvement and other mucosal sites such as conjunctiva , nasal passages and oesophagus were free of lesions .
intensely erythematous gingivae with multiple erosions erosive lesions on the gingiva palatally based on the history , multiple erosions and the apparent fragility of the gingivae experienced during examination , a vesiculobullous disorder was suspected .
is usually seen in the elderly , the former was thought to be more likely .
the other common conditions that can present with similar manifestations are oral lichen planus , drug hypersensitivity or idiopathic .
histopathological examination revealed suprabasal blister formation associated with extensive acantholysis of keratinocytes , suggestive of pv [ figure 3 ] .
photomicrograph showing suprabasilar split and acantholytic cells since the patient only had isolated gingival involvement , she was started on systemic corticosteroids ( prednisolone 0.5 mg / kg / day ) .
this initial dose was stepped up to 1mg / kg / day , which gave marked improvement in two weeks .
the patient was instructed to apply twice daily topical steroid oral paste ( triamcinolone acetonide 0.1% ) along with local anesthetic gel .
sometime after commencement of corticosteroid therapy , scaling and root planing was performed . over the past 1 year
currently , the patient is on this daily low - dose systemic corticosteroid therapy ( prednisolone ) , topical steroid oral paste ( triamcinolone acetonide 0.1% ) and supplementary medications . till date , no other sites are involved .
pemphigus is a group of potentially life - threatening autoimmune mucocutaneous diseases characterized by epithelial blistering affecting cuteneous and/or mucosal surfaces , the term being derived from the greek word pemphix ( bubble or blister ) .
although  vulgaris means common in latin , the worldwide incidence of pv is low and has been reported to be 0.10.5 per 100,000 persons per year .
pv is the most common variant of pemphigus , comprising of 80% of the disease entity .
pv frequently involves the mouth and has a fairly strong genetic background ; ethnic groups such as ashkenazi jews and people of mediterranean and indian origin are particularly susceptible and there is a link to hla class ii alleles .
clinically , pv appears to occur in males and females in an equal ratio , and is most frequently reported in patients between the fourth and sixth decades of life .
mortality from pv before the development of effective therapies was as high as 90% , mainly due to dehydration and secondary systemic infection . oral lesions are common and early manifestations of pv , seen typically in adults ( rarely in childhood ) .
one recent multicenter study in several countries showed that bulgarian patients less frequently had oral mucous membrane lesions ( 66% ) compared with italian ( 83% ) and israeli ( 92% ) patients .
initially vesiculobullous , the oral lesions readily rupture and as the older ones rupture and ulcerate , new bullae develop .
gingival lesions are less common and usually comprise severe desquamative or erosive gingivitis , characterized by red erosions or deep ulcerative craters . in the present case ,
gingiva was the only site involved ; the gingiva was intensely erythematous and erosive involving both attached and marginal gingiva .
however , the patient 's oral hygiene was very poor and this in turn contributed further to gingival inflammation , leading to generalized periodontitis .
the classic signs of oral or gingival pv are multiple erosions or desquamation and a positive nikolsky sign which both of which were present in our case .
histologically , there is an intraepithelial blister associated with acantholytic cells , features which were evident in this patient .
systemic corticosteroids are the treatment of choice in patients with pv ; topical steroid therapy alone is insufficient for sustained control of the disease because of the systemic autoimmune nature of pv . in the present case ,
systemic and topical corticosteroid treatment and adjuvant therapy of antifungal mouthwash , use of soft brush and vitamin supplementation were instituted .
pv is a chronic autoimmune mucocutaneous disease that often primarily involves the oral cavity . as it is a life - threatening disease condition , it is important for the clinician to be able to recognize oral manifestations of pv at an early stage and treat or refer appropriately .
dental professionals can thus play an important and significant role in the early diagnosis and management of pv .
 pemphigus vulgaris ( pv ) is an autoimmune blistering disease affecting the mucous membrane and skin . 
 typically , oral lesions appear before skin lesions , and in a majority of the cases only oral lesions are present . 
 the dentist may then be the first to recognize and diagnose this disease . 
 it is unusual for pv to present over the gingiva as a primary site of involvement . 
 diagnosis is based on clinical presentation and confirmed by histopathological study . 
 early diagnosis and management can prevent the uneven life- threatening effects of this potentially chronic mucocutaneous disorder . the case serves to enhance our awareness of the gingiva as a site at which systemic disease can manifest itself . 
INTRODUCTION
CASE REPORT
DISCUSSION
CONCLUSION