Bullous Pemphigoid Clinical Practice Guidelines (EADV, 2022)

Guidelines for the management of bullous pemphigoid were published in June 2022 by the European Academy of Dermatology and Venereology (EADV) in Journal of the European Academy of Dermatology and Venereology.^[1]

Diagnosis

Use indirect immunofluorescence (IIF) microscopy studies to detect circulating IgG anti-basement membrane zone (BMZ) autoantibodies; novel multivariant assays using multiple antigenic substrates are also recommended.

Use ELISA to detect anti-BP180 NC16A IgG autoantibodies and/or anti-BP230 IgG autoantibodies.

Obtain the biopsy specimen for direct immunofluorescence (DIF) studies from perilesional skin, defined as either erythematous non-bullous skin or normal skin within 1–2 cm from a lesion.

Treatment

Use clobetasol (CS) propionate 0.05% cream 20 to 30 g per day in mild-to-moderate disease and 30 to 40 g/day in extensive disease, initially administered once or twice a day, over the entire body including both normal skin and skin with blisters and erosions, but sparing the face until control of disease activity (CDA) has been achieved.

Once CDA is achieved, continue high-potency topical CS at the same dosage for 15 days, and then progressively taper it over a period between 4 months at the earliest, and 12 months.

After 4 months of treatment continue a maintenance treatment for 8 months and then stop.

Stop treatment within 4 months, in particular if BP activity has been rapidly controlled by topical CS and bullous pemphigoid (BP) is in remission.

Before discontinuing treatment in patients in remission, it may be useful to perform an ELISA-BP180.

In patients who relapse after treatment withdrawal, use 10 g daily of CS for patients with a localized relapse, 20 g CS daily for patients with mild disease, and 30–40 g daily for patients with moderate-to-extensive relapse.

A starting dose of 0.5 mg prednisone /kg daily is recommended in patients with mild/moderate and severe BP.

Discontinue treatment in patients who are free of symptoms for at least one to 6 months under minimal therapy with oral prednisone (0.1 mg/kg/day), clobetasol propionate (10 g/week), or immunosuppressants.

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Cite this: Bullous Pemphigoid Clinical Practice Guidelines (EADV, 2022) - Medscape - Aug 10, 2022.