BULLOUS PEMPHIGOID Date of Publication: June 20, 2017

Home , Bullous pemphigoid, Hives, Pemphigoid

Disease/Medical Condition

(also known as “BP”; includes “childhood bullous pemphigoid” and “pemphigoid gestationis”1)

Is the initiation of non-invasive dental hygiene procedures* contra-indicated? No (assuming patient/ client is already under medical care for BP).

 Is medical consult advised? — Yes, if suspect oral lesions have not yet been diagnosed. Biopsy is necessary for a definitive diagnosis. — Yes, if not already done, all patients/clients diagnosed with oral pemphigoid lesions should be referred to a dermatologist, as well as an ophthalmologist for assessment of ocular lesions. Examination by a gastroenterologist is advised for patients/ clients with dysphagia or severe oral lesions to detect possible involvement of the esophagus. — Yes, if after treatment and remission by a specialist, new lesions are observed. The dental hygienist should ensure referral back to the physician for re-evaluation and further treatment.

Is the initiation of invasive dental hygiene procedures contra-indicated?** Possibly, depending disease control and treatment regimen.

 Is medical consult advised? ...... See above.  Is medical clearance required? ...... Possibly (e.g., if the disease is unstable and/or oral involvement is severe). Also, medical clearance may be required if patient/client is being treated with medications associated with immunosuppression +/- increased risk of infection (e.g., systemic corticosteroids2 [i.e., intravenous formulations or oral prednisone], methotrexate, azathioprine, cyclophosphamide, cyclosporine, mycophenolate mofetil, and biologics [e.g., rituximab]). Thrombocytopenia (low platelet count) is a side effect of many non-steroidal immunosuppressive drugs, and medical clearance may be required to rule out significant bleeding risk.  Is antibiotic prophylaxis required? ...... No, not typically (although extended use of corticosteroids or cytotoxic drugs — particularly in the presence of leukopenia [low white blood cell count] — may warrant consideration of antibiotic prophylaxis).3  Is postponing treatment advised? ...... Possibly (depends on severity and level of control of the disease, as well as medical clearance for patients/clients on medications associated with immunosuppression and/or thrombocytopenia).

Oral management implications

 Oral prophylaxis should ideally be performed prior to the initiation of systemic or topical therapy.  Routine oral hygiene may be compromised due to pain in the mouth. During active oral disease, frequent patient/client follow-up is recommended (as frequently as every 4 to 6 weeks for debridement), and it should include monitoring for oral candidiasis. Gentle technique is important given the friability of oral tissues. During clinical remission, patient/client maintenance appointments should occur every 3 to 6 months, with as little disruption to oral tissue as possible.  Simple hand scaling instruments are often most effective, particularly if there is severe mucosal disease. If there is oral involvement, air polishers and abrasive products should be avoided due to the fragility of oral tissue. Gentle technique is important.

1 Pemphigoid gestationis is a very rare autoimmune bullous disease of pregnancy, and was formerly named herpes gestationis despite no connection with herpes virus infection. It is characterized by extremely itchy skin papules, as well as tense blisters. Mucosal involvement occurs in less than 20% of affected persons. 2 Compared with pemphigus, BP usually responds to lower doses of systemic corticosteroids. 3 While not intended as antibiotic prophylaxis for oral procedures, some patients/clients are prescribed antibiotics (with particular anti- inflammatory properties) as part of their pemphigoid treatment regimen, including dapsone, tetracycline, and erythromycin.

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Disease/Medical Condition

BULLOUS PEMPHIGOID

(also known as “BP”; includes “childhood bullous pemphigoid” and “pemphigoid gestationis”)

Oral management implications (cont’d)

 The dental hygienist should anticipate that the patient/client may experience pain and bleeding during procedures, and plan for this by scheduling extra time and using suction and gauze as needed.  Occlusive topical steroid therapy may be indicated in some patients/clients. This can be accomplished by using custom-made trays to localize steroid application.  For discrete ulcers, intralesional therapy with an injectable corticosteroid (e.g. dexamethasone) may be beneficial. A dentist would typically administer this in conjunction with the patient/client’s physician.  Intraoral photography during appointments can assist in evaluating oral tissue involvement.  Patients/clients with oral manifestations should be advised to eat a balanced diet and to avoid rough, acidic, and spicy foods. Alcohol (including in mouth rinses) is contraindicated. During flare-ups, a soft and bland diet is preferred in order to minimize trauma to injured tissue, although this can lead to more plaque accumulation. During periods of remission, patients/clients generally have no dietary restrictions.  The dental hygienist plays a role in monitoring the patient/client for long-term effects of corticosteroid and immunosuppressive treatment.

Oral manifestations

 Oral lesions are much less frequent than in mucous membrane pemphigoid (MMP), occurring in only a minority of patients/ clients, and usually only when cutaneous disease is extensive. However, in childhood bullous pemphigoid, the mucous membranes are much more commonly involved than in adults with BP.  When they occur, the oral lesions are very similar to those of MMP. Bullae and erosions appear, particularly on the attached gingiva. Other areas of the gingiva may also be affected, as well as the buccal mucosa, soft palate, and floor of the mouth. Non-gingival mucosal lesions tend to be more extensive and painful.  Some drugs used to treat pemphigoid, such as cyclosporine, have side effects that include bleeding, oral ulcerations, stomatitis, and tender or swollen gums. Methotrexate can cause mouth sores, in addition to a sore throat.

Related signs and symptoms

 Bullous pemphigoid is a chronic autoimmune disease, which manifests predominately as cutaneous lesions. Two-thirds of patients/clients only have skin lesions, with 1/3 having concomitant lesions of the mucous membranes. There is no definitive cure.  BP is the most common autoimmune mucocutaneous blistering disease, with an estimated prevalence of 1 in 40,000. Incidence is estimated to be 7 to 10 new cases per million persons per year. BP tends to affect older persons more than mucous membrane pemphigoid (MMP), with no pronounced gender predilection. Peak incidence is in the seventh and eighth decades.  Triggering factors can be identified in up to 15% of patients/clients. These include trauma (such as surgical procedures, burns, and ionizing radiation), infections, and occasionally certain medications (such as furosemide [diuretic], carbamazepine [anticonvulsant], captopril [antihypertensive], and antibiotics [amoxicillin, penicillin, and ciprofloxacin]).  Similar to MMP, BP lesions result from cleavage of the epithelium from underlying connective tissue at the basement membrane, without the acantholysis4 found in pemphigus vulgaris. Tense blisters are more characteristic of pemphigoid than of pemphigus. Unlike MMP, the blisters of BP usually heal without scarring.

4 Acantholysis is breakdown of cellular adhesion between epithelial cells, which results in intraepithelial vesicle formation.

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Disease/Medical Condition

BULLOUS PEMPHIGOID

(also known as “BP”; includes “childhood bullous pemphigoid” and “pemphigoid gestationis”)

Related signs and symptoms (cont’d)

 Skin lesions typically occur on the trunk and limbs (although the whole body may be affected), often starting with a very itchy, red papular rash that may be mistaken for urticaria (hives). This non-bullous, prodromal rash may recur for several months, eventually evolving into areas that form vesicles and bullae that then break and leave sore, open skin.  In childhood bullous pemphigoid in infants younger than 1 year of age, the palms and soles are often affected by blisters.  While mortality does occur, this chronic disease is usually not life threatening. It can be a serious condition, particularly in the elderly. Periods of exacerbation may be interspersed with periods of remission. BP can be self-limited, resolving after many months to years, even without therapy.  Osteoporosis, glaucoma, cataracts, type 2 diabetes mellitus, and infections are complications associated with long-term systemic corticosteroid therapy (e.g., prednisone).

References and sources of more detailed information

 Canadian Skin Patient Alliance http://www.canadianskin.ca/en/pemphigoid-and-pemphigus  Medscape — Oral Manifestations of Autoimmune Diseases http://emedicine.medscape.com/article/1077969-overview  Canadian Pemphigus and Pemphigoid Foundation http://www.dermatology.ca/wp-content/uploads/2012/01/CPPF-brochure-EN.pdf  Cleveland Clinic http://www.clevelandclinicmeded.com/medicalpubs/diseasemanagement/dermatology/blistering-diseases/  Mayo Clinic http://www.mayoclinic.org/diseases-conditions/bullous-pemphigoid/home/ovc-20157298  International Pemphigus and Pemphigoid Foundation http://www.pemphigus.org/persistent-mouth-sores-working-with-your-oral-health-care-provider-to-reach-a-diagnosis/ http://www.pemphigus.org/research/clinically-speaking/pemphigoid/ http://www.pemphigus.org/research/clinically-speaking/treatments/ http://www.pemphigus.org/research/clinically-speaking/complications-side-effects/ http://www.pemphigus.org/research/clinically-speaking/diagnostic-testing/ http://www.pemphigus.org/research/clinically-speaking/risk-factors/ http://www.pemphigus.org/research/clinically-speaking/current-consensus-of-disease-outcomes/ http://www.pemphigus.org/research/clinically-speaking/prevention-and-psychology/  Patient http://patient.info/doctor/pemphigoid-gestationis  DermNet New Zealand https://www.dermnetnz.org/topics/childhood-bullous-pemphigoid  Canadian Pemphigus http://pemphigus.ca/about-pemphigoid/

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Disease/Medical Condition

BULLOUS PEMPHIGOID

(also known as “BP”; includes “childhood bullous pemphigoid” and “pemphigoid gestationis”)

References and sources of more detailed information (cont’d)

 Ibsen OAC and Phelan JA. Oral Pathology For The Dental Hygienist (6th edition). St. Louis: Elsevier Saunders; 2014.  Regezi JA, Sciubba JJ, and Jordan RCK. Oral Pathology: Clinical Pathologic Correlations (6th edition). St. Louis: Elsevier Saunders; 2012.  Little JW, Falace Da, Miller CS and Rhodus NL. Dental Management of the Medically Compromised Patient (8th edition). St. Louis: Elsevier Mosby; 2013.

* Includes oral hygiene instruction, fitting a mouth guard, taking an impression, etc. ** Ontario Regulation 501/07 made under Dental Hygiene Act, 1991. Invasive dental hygiene procedures are scaling teeth and root planing, including curetting surrounding tissue.

Date: February 16, 2017