A new set of guidelines from Japan regarding the management of bullous pemphigoid, including epidermolysis bullosa acquisita and mucous membrane pemphigoid have been published in the Journal of Dermatology.
To identify the severity of bullous pemphigoid, the guideline committee recommends using the Bullous Pemphigoid Disease Area Index (BPDAI) score. Local topical therapy plus tetracycline, nicotinamide, diaminodiphenyl sulfone, or a low-dose steroid is recommended in mild cases. In moderate and severe cases of bullous pemphigoid, the guideline recommends moderate- or high-dose steroid therapy plus an immunosuppressant, steroid pulse therapy, intravenous immunoglobulin (Ig) therapy, or plasma exchange therapy if efficacy is not achieved with a standard approach.
In mucous membrane pemphigoid, the assessment of disease activity can be conducted using the BPDAI or MMPDAI at the time of treatment. Treatment is based on clinical manifestations. Patients in the low-risk group who have localized lesions only on the skin and oral mucosa should be treated with topical steroid therapy and sometimes topical steroids plus tetracycline or minocycline and nicotinamide. Patients in the high-risk group with several oral mucosal lesions; advanced oral mucosal lesions; or ocular, genital, esophageal, or laryngeal mucosal lesions should be treated with oral steroids. Patients with high-risk mucous membrane pemphigoid may also find intravenous Ig therapy and plasma exchange useful, the committee wrote. In cases of relapse, the guideline recommends reinitiating treatment with 1.5 to 2 times the steroid dose. At this stage, clinicians may wish to consider concomitant use of immunosuppressants.
In bullous pemphigoid, the guideline recommends systemic steroid administration as standard treatment (degree of recommendation, A). Concomitant use of intravenous Ig therapy may be considered in patients with severe bullous pemphigoid who do not respond to conventional therapy (degree of recommendation, B). In addition, concomitant use of oral steroids and plasma exchange or azathioprine or mizoribine may be useful for reducing clinical symptoms and reducing the dose of oral steroids. Caution is warranted for the concomitant use of oral steroids and azathioprine, however, due to the risk for liver dysfunction.
Similar to bullous pemphigoid, systemic steroid therapy is also recommended for mucous membrane pemphigoid (degree of recommendation, A). Oral cyclophosphamide, in addition to oral steroids, may be useful as first-line therapy, particularly in severely progressive ocular and mucosal lesions (degree of recommendation, B). Systemic steroid administration is also deemed useful for epidermolysis bullosa based on committee opinion (degree of recommendation, B).
“Although these guidelines represent the current, standard medical treatment guidelines in Japan,” the committee wrote, “in actual clinical practice, it is necessary to treat patients with full consideration of the diverse backgrounds of individual patients.”
Ujiie H, Iwata H, Yamagami J, et al; Committee for Guidelines for the Management of Pemphigoid Diseases (Including Epidermolysis Bullosa Acquisita). Japanese guidelines for the management of pemphigoid (including epidermolysis bullosa acquisita). J Dermatol. 2019;46(12):1102-1135.