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Facing the bullous: Helping patients in their fight against oral pemphigus

June 30, 2020
Pemphigus vulgaris, a potentially life-threatening autoimmune disorder, often first presents in the oral cavity. Dental hygienists should familiarize themselves with the signs and treatments.

Pemphigus vulgaris (PV) is part of a group of autoimmune disorders that affects the mucous membrane and skin. It causes bullouses of the skin, and if not treated early, can be life threatening.1 In cases of oral PV, the immune system looks for proteins that bind to the cells of the skin, which “causes a buildup of fluid between the skin cells, resulting in blisters.”2 In the majority of cases, the lesions present themselves first in the oral cavity followed by other areas in the body. This is why it is crucial for dental hygienists to familiarize themselves with the disorder because we may be the first ones to help patients get the treatment they need.3

Just like a blister that occurs anywhere else on the body, the lesions found in oral PV fill up and then burst. This causes their appearance to change constantly, so intraoral cameras and detailed chart notes can be invaluable in these cases. Hygienists should be comfortable communicating with other health-care providers who are treating the patient, such as ophthalmologists, dermatologists, oral pathologists, and oral surgeons. Sharing chart notes, biopsy reports, and intraoral images with the patient’s care team ensures the patient gets the comprehensive treatment needed. You can view images of the lesions here and here.

As hygienists, we understand the importance of a thorough intraoral exam and that they have the potential to save lives. While performing our exam on a patient with oral pemphigus, it is important to be aware that the lesions are most commonly found on the buccal mucosa, palate, and lips.3

Even if bullouses aren’t present, the tissues can appear fragile and easily damaged. The blister-like lesions themselves can be both erythematous and erosive and can cause patients extreme pain. This can lead to a decrease in their ability to maintain good oral hygiene, which in turn can lead to gingivitis and later, periodontal disease. This can be a vicious, discouraging cycle for patients. As hygienists, it is our job to encourage patients to persevere and come up with a home-care plan that is easy, effective, and comfortable.

When treating a patient with PV, it is important for hygienists to have a gentle and delicate touch, since the tissues can be easily damaged. Hygienists should consider avoiding abrasive prophy pastes, alcohol-containing products, and anything else that may cause further irritation or become lodged in tissues. We may have to think outside of the box and familiarize ourselves with different products and home-care alternatives. We should choose products and techniques that allow patients to maintain the best oral hygiene as possible while minimizing their discomfort. For example, infant toothbrushes, disposable oral swabs, and chewing gum could all be recommended as alternatives to traditional toothbrushes. Patients should be discouraged from using aggressive brushing methods and electric toothbrushes when lesions are present. 

Nutritional counseling should also be considered, and patients should be advised to avoid irritating food and drinks, (e.g., very hot dishes and drinks, overly spicy or acidic foods, and alcohol). Food and drinks that easily dehydrate the skin (e.g., those containing caffeine) may also be considered contributing factors. Since these patients cannot usually perform good oral hygiene, food and drinks that are high in sugars should also be discussed because they can lead to an increased risk for caries. Because of the discomfort, these patients may choose foods that fall in a soft diet category, and may have difficulty achieving good overall health.4

Both systemic and topical steroids can be courses of treatment for managing oral PV as well as certain immunosuppressive drugs. Topical anesthetics to provide the patient comfort may also be indicated.5 

Patients with oral PV need to be closely monitored for changes to their tissues and for advancement in the disease. If the patient has periodontal disease, it is a good practice to see him or her every three to four months for a periodontal maintenance recall, at which point an oral evaluation would be performed in conjunction with each visit. If the patient doesn’t have periodontal disease, they should still be seen regularly by their dentist or oral surgeon for an evaluation of the oral cavity.

It is important to note that the only definitive way to diagnose a patient with oral PV is with a biopsy. A biopsy must be performed in order to rule out other possible erosive conditions, such as lichen planus.

The International Pemphigus & Pemphigoid Foundation is a great resource for our patients suffering with this disorder. The website has great content, a health-care provider locator tool, but also has encouraging patient stories that may be able to help them cope with their diagnosis. The website also has a wealth of information for us dental hygienists. There are continuing education articles, photos, and even a place to sign up to join their team of ambassadors to help promote education about the disorder which in turn will help patients get a diagnosis quicker.

References

  1. Pettini F, Ballini A, Capodiferro S, et al. Management of oral pemphigus vulgaris: a case report and a clinical update. Eur J Inflamm. 2015:13(1):53-57. doi:10.1177/1721727X15576854
  2. Pemphigus vulargis. Johns Hopkins Medicine. https://www.hopkinsmedicine.org/health/conditions-and-diseases/pemphigus-vulgaris
  3. Kumar SJ, Nehru Anand SP, Gunasekaran N, Krishnan R. Oral pemphigus vulgaris: A case report with direct immunofluorescence study. J Oral Maxillofac Pathol. 2016;20(3):549. doi:10.4103/0973-029X.190979
  4. Gambino A, Carbone M, Arduino PG, Carcieri P, Carbone L, Broccoletti R. Conservative approach in patients with pemphigus gingival vulgaris: a pilot study of five cases. Int J Dent. 2014;2014:747506. doi:10.1155/2014/747506
  5. Samundeeswari S,  Ramesh S, B Manimegalai. Role of intensive nutrition support in improving serum albumin level and its outcome on healing skin lesions in a pemphigus vulgaris patient: a case report. International Journal of Advance Research, Ideas and Innovations in Technology. 2018;4(6):482-485. https://www.ijariit.com/manuscripts/v4i6/V4I6-1298.pdf