Sections

Treatment

Medical Care

The aim of treatment in pemphigus vulgaris is the same as in other autoimmune bullous diseases, which is to decrease blister formation, promote healing of blisters and erosions, and determine the minimal dose of medication necessary to control the disease process. Therapy must be tailored for each patient, taking into account preexisting and coexisting conditions. Patients may continue to experience mild disease activity while under optimal treatment.^{[38, 39, 40, 41]}

See the images below.

Erosions and healing areas on the back.

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Healing areas on the chest and abdomen.

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Corticosteroids have improved overall mortality, but now much of the mortality and morbidity in these patients relates to the adverse effects of therapy. Whether massive doses of steroids have any advantage over doses of 1 mg/kg/d is unclear.^[42]

Immunosuppressive drugs are steroid sparing and should be considered early in the course of the disease. Epidermal growth factor may speed healing of localized lesions.^[43] Many authorities now use rituximab, the anti-CD20 antibody, as first- or second-line therapy, with evidence from 2017 suggesting better outcomes when rituximab is used as a first-line agent.^{[44, 45, 46, 47, 48, 49, 50, 51, 52]}

Rituximab was approved by the US Food and Drug Administration (FDA) for treatment of pemphigus vulgaris in June 2018. Approval was based on a randomized trial that compared rituximab plus oral corticosteroid treatment with corticosteroid treatment alone as first-line therapy for patients with moderate-to-severe pemphigus vulgaris.^[52]At month 24, 41 (89%) of 46 patients assigned to rituximab plus short-term prednisone were in complete remission off-therapy compared with 15 (34%) of 44 assigned to prednisone alone (P< .0001). Targeted B-cell depletion is promising.^[53]

The antitumor necrosis factor drugs sulfasalazine and pentoxifylline have been reported as effective adjunctive treatments, reducing the serum level of tumor necrosis factor and resulting in rapid clinical improvement.^[54]Methotrexate has also been used.^{[55, 56]}Dapsone has been suggested as a steroid-sparing agent in the maintenance phase of pemphigus vulgaris treatment^{[57, 58]}; dapsone has also been suggested as a first-line agent.^[59]

Intravenous immunoglobulin therapy has been suggested as efficacious in pemphigus vulgaris treatment.^{[60, 61, 62, 63]}Amagai et al reported on the successful use of intravenous immunoglobulin in pemphigus patients who did not fully respond to systemic steroids,^[64]and Asarch et al reported its use in pediatric patients.^[65]

Infliximab has proven effective in some patients with refractory disease, and photodynamic therapy has been suggested as a possible adjunctive treatment for recalcitrant ulceration.^[66]Mizoribine has been used for refractory ocular manifestations.^[67]

Plasmapheresis has been used in refractory cases, usually in conjunction with cytotoxic therapy.^{[68, 69]}

Consultations

Management of patients with pemphigus vulgaris requires coordination of care between the dermatologist and the patient's primary care physician.

An ophthalmologist should evaluate patients with suspected ocular involvement and those requiring prolonged high-dose steroids.

Patients with oral disease may require a dentist and/or an otolaryngologist for evaluation and care.

Patients on systemic steroids should maintain adequate vitamin D and calcium intake through diet and supplements. Patients with a history of renal calculi should not receive calcium carbonate.

Patients receiving long-term systemic corticosteroids should be evaluated by a rheumatologist within the first 30 days of treatment for osteoporosis risk assessment and consideration of a bisphosphonate for prophylaxis against osteoporosis.

Diet

No dietary restrictions are needed, but patients with oral disease may benefit from avoiding certain foods (eg, spicy foods, tomatoes, orange juice) and hard foods that may traumatize the oral epithelium mechanically (eg, nuts, chips, hard vegetables and fruit).

Activity

Advise patients to minimize activities that traumatize the skin and that may precipitate blistering, such as contact sports. Nontraumatic exercises, such as swimming, may be helpful. Additionally, dental plates, dental bridges, or contact lenses may precipitate or exacerbate mucosal disease.

Complications

Secondary infection, which may be either systemic or localized to the skin, may occur because of the use of immunosuppressants and the presence of multiple erosions. Cutaneous infection delays wound healing and increases the risk of scarring.

Long-term immunosuppressant therapy may result in infections and secondary malignancies (eg, Kaposi sarcoma), owing to impaired immune surveillance.

Growth retardation has been reported in children taking systemic corticosteroids and immunosuppressants.

Bone marrow suppression has been reported in patients receiving immunosuppressants. An increased incidence of leukemia and lymphoma is reported in patients receiving prolonged immunosuppression.

Impaired immune responsiveness caused by corticosteroids and other immunosuppressive drugs may result in the rapid spread of infection. Corticosteroids suppress clinical signs of infection and may allow diseases such as septicemia or tuberculosis to reach an advanced stage before diagnosis.

Osteoporosis may occur following the use of systemic corticosteroids.

Adrenal insufficiency has been reported following prolonged use of glucocorticoids.

Medication

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Media Gallery

Early, small blister filled with clear fluid arises on healthy skin.
Flaccid blister filled with clear fluid arises on healthy skin.
An erosion.
Erosions and healing areas on the back.
Healing areas on the chest and abdomen.
Direct immunofluorescence showing intercellular immunoglobulin G throughout the epidermis of a patient with pemphigus vulgaris.

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Author

Bassam Zeina, MD, PhD Consulting Staff, Department of Dermatology, Milton Keynes Hospital, UK

Bassam Zeina, MD, PhD is a member of the following medical societies: British Medical Association, Royal Society of Medicine, British Association of Dermatologists

Disclosure: Nothing to disclose.

Coauthor(s)

Nicole Sakka, MBBS Foundation Year 2, Royal Liverpool and Broadgreen University Hospital, Liverpool, UK

Disclosure: Nothing to disclose.

Sohail Mansoor, MBBS, MSc Dermatologist and Lead Physician in Dermatologic Surgery, Department of Dermatology, Barnet Hospital, UK

Sohail Mansoor, MBBS, MSc is a member of the following medical societies: American Academy of Dermatology, American Society for Dermatologic Surgery, Royal College of Physicians, American Academy of Anti-Aging Medicine, Royal College of Physicians and Surgeons of Glasgow

Disclosure: Nothing to disclose.

Specialty Editor Board

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, American College of Rheumatology

Disclosure: Received income in an amount equal to or greater than $250 from: Biogen US (Adjudicator for study entry cutaneous lupus erythematosus); Priovant (Adjudicator for entry into a dermatomyositis study); IQVIA (Serono - adjudicator for a study of cutaneous LE) <br/>Received honoraria from UpToDate for author/editor; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for these trust accounts for: Stocks held in various trust accounts: Allergen; Amgen; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble;; Celgene; Gilead; CVS; Walgreens; Bristol-Myers Squibb.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Abby S Van Voorhees, MD Assistant Professor, Director of Psoriasis Services and Phototherapy Units, Department of Dermatology, University of Pennsylvania School of Medicine, Hospital of the University of Pennsylvania

Abby S Van Voorhees, MD is a member of the following medical societies: American Academy of Dermatology, Women's Dermatologic Society, National Psoriasis Foundation, American Medical Association, Phi Beta Kappa, Sigma Xi, The Scientific Research Honor Society

Disclosure: Received honoraria from Amgen for consulting; Received honoraria from Abbott for consulting; Partner received salary from Merck for management position; Received honoraria from Abbott for speaking and teaching; Received honoraria from Amgen for review panel membership; Received honoraria from Centocor for consulting; Received honoraria from Leo for consulting; Received none from Merck for other.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author, Mohsin Ali, MBBS, FRCP, MRCP, to the development and writing of this article.

Pemphigus Vulgaris Treatment & Management

Medical Care

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Activity

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