Pemphigus Vulgaris

Figure 19. Pemphigus vulgaris – clinical.
Figure 20. Early diagnosis and treatment of PV.
Figure 21. PV histology and DI.
Figure 22. Immunofluorescence of Pemphigus Vulgaris showing IgG in the intracellular spaces of the epithelium and cellular separation.
Figure 23. PV with severe epithelial slough.
Figure 24. The “key” for PV-early diagnosis and treatment.

Pemphigus is a rare, heterogeneous group of idiopathic, autoimmune vesiculo-bullous diseases of skin and mucous membranes that are characterized by development of antibodies to desmoglein (dg) 1 or 3, the intrarcellular transmembraneous glycoproteins involved in maintenance of adhesion between epithelial cells of the skin and mucous membranes. Dg3 is the predominant antigen found in oral PV, while dg 1 and 3 are both elevated when the skin is affected. An interesting case series described successful topical treatment of mild oral PV associated only with dg3 but subsequently the condition recurred involving both skin and oral mucosa and lesions were accompanied by a transition to both dg1 and dg3 antigen.32,35 Pemphigus vulgaris (PV) is the most common and severe form of the pemphigus disease group. It is potentially life-threatening and it causes various degrees of acantholysis in the epithelium, creating blistering lesions on mucosa and skin. PV can occur at any age and it affects males and females equally.10,15,31,102 On skin, PV can involve multiple tissue surfaces leading to potential septicemia and loss of tissue fluid and electrolytes. Untreated PV was once considered to be invariably fatal. Fortunately several highly effective therapeutic techniques have been developed that have markedly reduced the incidence of fatality.30,102

PV often affects mucous membranes and can have devastating effects there as well and oral lesions are very common. Indeed, oral lesions are the first site of PV involvement in 50%-80% of patients, and early diagnosis and treatment can often minimize disease progression.32,35

Unfortunately, a recent survey sponsored by the International Pemphigus and Pemphigoid Foundation (IPPF) determined the average time from onset to diagnosis of PV and MMP is approximately 6 months or longer.86 Consequently, the condition may be in an advanced stage before therapy is initiated. This fact may be of special importance for the dental provider since the first PV lesion typically occurs in the mouth and the symptomatic patient will usually seek care from their general dentist or periodontist. Dental health care providers are normally not responsible for the treatment of PV, rather they serve to facilitate diagnosis with biopsy evaluation of early lesions or referral for biopsy. Disease therapy is commonly provided by dermatologists, otolaryngologists or internists after referral from the dental practice.

PV lesions can occur on any oral mucosal surface. The buccal mucosa is involved most often followed by the gingiva and tongue. Gingival lesions often present as DG and the gingiva may be the only site of lesions early in the disease process.58

Oral PV rarely responds to topical corticosteroid therapy, although intralesional steroid injections are sometimes of benefit for oral refractory sites other than the gingiva.32,35 Systemic therapy can usually be best managed by a dermatologist, otolaryngolgist, or internist. Short- or long-term systemic corticosteroids are often prescribed, while steroid sparing therapy may be employed using aziathioprine, dapsone, mycofenolate mofetil, or others, and plasmapheresis or IV immune globulin has been utilized. At present, the monoclonal antibody rituximab is increasingly being used with reports of favorable results.44,101

Several reports of DG related PV lesions have described increased frequency and severity of dental and periodontal diseases in PV patients with gingival or other oral lesions.92 However, the use of the conservative minimally invasive oral hygiene measures previously described may help maintain oral health during acute PV phases while gentle oral hygiene measures may be of significant benefit once the oral lesions have improved or reached remission. Although some have suggested the use of a powered toothbrush, our findings indicate sonic and powered toothbrushes may occasionally worsen DG in patients with PV because the brushes disrupt the epithelial mucosal layer rather easily.92 As with other idiopathic autoimmune diseases, the lesions may occasionally recur after remission and the patient must be instructed to seek care immediately should that happen. A maintenance recall interval of approximately 3 months is often recommended for the first 1-2 years following disease resolution.