Disease Management

Prevention and Other Aspects of Management

Several factors seem to exacerbate pemphigus, including dental work, sun exposure, X-rays and trauma.

Oral issues:

Prophylactic administration of 20mg per day of prednisone prior to dental work has been suggested (o).
For oral pemphigus, measures such as soft diets and soft toothbrushes help minimize local trauma. Topical analgesics or anesthetics are useful in alleviating oral pain.
Oral hygiene is crucial. Otherwise PV may be complicated by dental decay; toothbrushing should be encouraged and antiseptic mouthwashes may be used, such as chlorhexidine gluconate 0.2% or 1:4 hydrogen peroxide solutions.
Patients are also susceptible to oral candidiasis, which should be treated.
Pemphigus can involve the esophagus, and nasal cavity leading to erosive esophagitis and occasionally significant bleeding.

Skin issues:

Minimize sun exposure, which can cause disease to flare. Encourage the use of sunscreens and protective clothing.
As non-healing lesions may be secondarily infected with bacteria, fungi, herpes, or wart viruses, have patients report any signs of skin or blister infection, herpetic lesions.

Dental Management

PV/MMP patients often require special care during maintenance appointments to manage sensitive areas of the mouth and prevent future blisters from forming. Open PV and MMP blisters can be very painful for patients, making it difficult to brush teeth and requiring a diet of soft foods. Consequently, patients may experience increased incidence of dental caries and periodontal disease.1 Presence of oral ulcerations may result in pain and bleeding during oral prophylaxis. However, this should not be a reason to delay or avoid prophylaxis, which should be done on a regular basis, even when lesions are present. Good oral hygiene is very important to positive treatment outcomes. Poor oral hygiene with dental plaque and gingival inflammation may reduce and delay treatment outcome.

Tips for dental professionals include:

Conduct a complete oral mucosal examination. Evaluate for any abnormalities, including oral complications of PV/MMP treatment, such as candidiasis.
Be gentle during maintenance appointments, and schedule more frequent appointments to control plaque build-up. 1,2
Awareness of CAMBRA (Caries Management by Risk Assessment) protocols since the soft diet and difficulties in home care may increase risk for caries.
Expect that the patient may experience pain and bleeding during the procedure. Plan for it by scheduling extra time and using local anesthetics, suction, and gauze as needed.
Use simple hand scaling instruments to increase control and disrupt the tissue as little as possible. 1,2
Polish teeth with a nonabrasive toothpaste, avoiding harsh abrasives and air polishers. 1
Provide a list of rinses not containing flavoring agents, alcohol, or other harsh ingredients that might be irritating to oral tissues.
Oral hygiene instructions for home care may need to be tailored based on the level of mucosal involvement. When significant oral disease is present, gentle home care including extra-soft toothbrushes, mildly flavored toothpastes, and mild mouth rinses may be all that the patient can tolerate. Some patients may not be able to floss due to bleeding and pain. During this time, frequent dental cleanings become more important. Once the oral ulcerations and associated pain decrease, the standard home care routine, including regular brushing and flossing, can be recommended.
If patients are on a soft diet due to presence of oral ulceration and pain, suggest nutritious options such as vegetable soups, fruit smoothies, etc.
If, after treatment and remission by a specialist, new lesions are observed, the general dentist should refer back to the specialist for re-evaluation and further treatment.

1. Burkhart N. Oral pemphigus vulgaris. Available at: www.rdhmag.com/articles/print/volume-27/issue-4/columns/oral-exams/oral-pemphigus-vulgaris.html.

2. Burkhart N. Mucous membrane pemphigoid. Available at: www.rdhmag.com/articles/print/volume-27/issue-3/columns/oral-exams/mucous-membrane-pemphigoid.html.

Psychosocial Issues

There is a paucity of knowledge about psychosocial issues in bullous conditions because of limited research and methodological problems in others. However, dermatologic conditions are commonly associated with psychiatric sequellae, and their course of may be significantly influenced by stress, emotional disturbances, or psychiatric disorder.

Information about psychosocial issues from other chronic complex conditions can safely be applied to the bullous conditions. In general, patients who face the challenge of chronic, unpredictable, and potentially embarrassing conditions which have a relapsing and remitting course suffer under a recurrent and persistent psychosocial burden. For example, patients with chronic heart failure have a high prevalence of anxiety and depression (about 40%), and moreover, the occurrence of depression and social isolation predicted mortality independent of demographic and clinical status. Further, in chronic obstructive pulmonary disease (COPD), data exists to show that interventions which reduce depressive symptoms affect COPD outcomes, and may improve survival, quality of life, self-management behavior and reduced health care utilization.

In addition, complex treatment with many medications taken several times a day, many of which have side effects that can range from weight gain and acne to more severe effects such as bone disease, high blood pressure, cataracts, diabetes, pancreatitis, suppression of the immune system, and increased cancer risk adds an additional set of concerns.

Intervention

Although many of the interventions for managing such psychosocial conditions are nonspecific (and intuitive) their potential effect should not be underestimated. Interventions can simply involve empathic listening and inquiry, education of the patient about the condition and its characteristics, challenging distorted or overly helpless thoughts and self-conceptions, and facilitating social contact and functioning. Patients with chronic complex conditions rate the following psychosocial aspects as highly important:

Good communication with their doctor
Understanding their medication
Dealing effectively with pain
Understanding the effects of their condition on their energy, their future, and their work

In one small study of pemphigus vulgaris, SF-36 (scores showed a decrease of mean scores in all SF-36 dimensions, except for physical pain and alteration in general status of health. The greatest alteration concerned the impact on physical and emotional status (p<0.00001) and the smallest alteration concerned the perception of general health (p=0.02). The factors influencing this impact were: profession, face involvement and extent of lesions.

Areas of Emphasis

Specific areas of emphasis are:

Stress and Coping: Stressful life events can be associated with symptom exacerbation. Problem-solving coping strategies and social support can lower psychological distress and improve quality of life.
Physical and Psychosocial Functioning: Patients with chronic conditions report lower quality of life than the general population. Everyday tasks such as work, travel, meetings and chores take on an additional layer of difficulty.
Body Image and Self-Esteem: Concerns about appearance can impact a patients sense of self esteem and capacity for enjoyment and achievement, and restrict patient’s social activities.
Needs of Caregivers: Caregiving is not always a choice, and dependency can be exaggerated in some areas where limited availability of social and community services exist. The caregiver often assumes responsibility for making weighty decisions about short-term care following a crisis, long-term care, and end-of-life care.
Family Counseling: Early recognition of issues and support for the patient and their families can be important.
Relationship Development: Social support is an important aspect of coping with chronic illness and intimacy, feeling connected to others who share similar experiences, communicating and networking with others, and having access to family, friends, and health care providers who can care for emotional and physical needs.
Lifestyle Changes: Assessing a person’s role in the family, workplace, and among friends helps to establish a baseline by which to measure future changes in role. For example, people who are accustomed to a leadership role can have more difficulty coping with a disease affects them, and this can force people out of the workplace and leisure activities, causing social isolation.
Age Related Issues: Each age group brings certain social stresses that should be considered in light of the patient’s condition. For example, adolescents may have significant image concerns, versus an elderly patient’s concern about isolation and anxiety.

Psychosocial Interventions

General psychosocial interventions in the setting of chronic illness include the following aspects. Achieving each of these interconnected concepts requires creativity, patience and repetition.

Recognition of Depression and Anxiety: Both depression and anxiety are common in chronic conditions and are under reported and under diagnosed. Depression is one of the leading causes of disability worldwide, and is associated with detrimental effects on role and physical function even when controlling for comorbid medical conditions.
Older adults with depression report greater functional impairment than those without depression, and this impairment persists over time. Encouraging openness about mood disorders and communication to others including health care providers is an important intervention.
Goal Setting: Provide attainable, short-term goals that step the patient towards a sense of self-esteem, self-efficacy, and self-care.
Condition Education: Learning to take an active role in one’s own healthcare (or for a caregiver) by better understanding the condition, its course and its treatment gives patients a strong sense of confidence and control, and helps facilitate dialog with their health care team. Understanding that others have had similar problems similarly enables patients to address these issues for themselves.
Community Resources: These should be evaluated for both the patient and caregiver (if any).