Updates on the Management of Autoimmune Blistering Diseases

Joanna N. Hooten, MD; Russell P. Hall 3rd, MD; Adela R. Cardones, MD


Skin Therapy Letter. 2014;19(5) 

In This Article

Approach to Patients With AIBD

Several factors need to be considered in treating a patient with an AIBD (Table 1). A complete history and thorough physical examination of the skin and mucous membranes must be performed. A skin biopsy, with direct and indirect immunofluorescence analysis, is important in arriving at the correct diagnosis and planning therapy. Determining titers of autoantibodies are not always necessary to confirm the diagnosis, but may be helpful in following the patient's progress.[2–4]

First, an accurate diagnosis will help determine the likelihood of disease remission, potential for mucous membrane involvement, risk of scarring, and other long-term sequelae that may affect treatment plans. For example, bullous pemphigoid (BP) and EBA may demonstrate a similar clinical presentation, but have a different natural history. A clinician may plan to wean a BP patient off all medications in 9–12 months, whereas longer-term therapy is often needed in patients with EBA.

Second, severity and extent of disease has to be carefully evaluated. The extent of the disease can be variable in all types of AIBD. Additionally, the definition of mild, moderate and severe disease differs among experts. Some authors define limited disease as <10% body surface area, while others use the cut-off point of <10 new blisters per day to delineate between limited and severe disease.[5,6] Nevertheless, even in the absence of new blisters and regardless of affected areas, the involvement of functional critical sites (e.g., hands and feet or mucosal surfaces) may require more aggressive therapy. Ocular disease, which can result in permanent scarring or blindness, warrants systemic treatment, in addition to subspecialty referral to ophthalmology.

Third, the presence of comorbidities may dictate the type and dosage of medication that can be used and must be accompanied by careful assessment. Diseases such as diabetes mellitus, hypertension, chronic infections (e.g., hepatitis or HIV), and previous or existent malignancies need to be considered. Patients who would otherwise be treated aggressively, but whose comorbidities preclude therapy with systemic corticosteroids (i.e., elderly patients or those with uncontrolled diabetes or hypertension) or more traditional steroid sparing agents, may instead have to be treated with topical, antibiotic or antiinflammatory medications.

Finally, the choice of medications may be restricted by side effects experienced by the patient. Potential side effects, which can be significant, include alterations in mental status, sleep disturbances, and gastrointestinal (GI) discomfort. The assessment of all adverse effects throughout therapy is critical to treatment success in AIBD. The clinician must exercise judgment in weighing the risks and benefits of initial therapy in an effort to maximize efficacy while minimizing systemic toxicity.