Epidermolysis bullosa acquisita (EBA) is a chronic subepidermal autoimmune bullous disease (sAIBD). Anticollagen 7 (COL-7) antibodies play central pathogenic roles in EBA, initiating inflammation and activating neutrophils to produce multiple cytokines that contribute to blister formation.¹ Standard therapy includes systemic and/or topical glucocorticoids with colchicine or dapsone, but the outcome is sometimes unsatisfactory.¹ Tofacitinib is a Janus kinase (JAK) inhibitor and blocks the cytokines network and immune progression through the JAK signal transducer and activator of transcription (STAT) pathway. In this article, we describe a patient with recurrent EBA who was treated with tofacitinib.

A 58-year-old man presented to our clinic with blisters and erosions on the proximal limbs and front trunk. The patient was otherwise healthy, without any other disorders. Biopsy results showed a subepidermal blister, and direct immunofluorescence results revealed linear deposition of immunoglobulin (Ig) G along the basement membrane zone (BMZ). Coupled with positive anti–COL-7 IgG, the patient received a diagnosis of inflammatory EBA. Prednisolone, 24 to 40 mg, daily, and methotrexate, 10 to 15 mg, weekly, were prescribed and tapered for a year but were discontinued due to pneumocystosis pneumonia and osteoporosis. During antiinfection treatments, dapsone and topical glucocorticoids were administered with minimal benefit. After the patient recovered from pneumocystosis pneumonia, the rash recurred on his thighs and buttocks (Figure, A) with COL-7–specific IgG of 165 U/mL (enzyme-linked immunoassay, MBL Co Ltd) and absolute neutrophil counts of 5.9 × 10³/L (normal range, 1.8-6.3 × 10³/L; to convert to × 10⁹/L, multiply by 0.001). Tofacitinib, 5 mg, twice daily, and prednisolone, 8 mg, daily, were then prescribed, and within 1 month, the patient noted that his EBA was improved (Figure, B). After 4 months of treatment, the blisters and erosions almost disappeared, with decreased COL-7-specific IgG levels (58 U/mL) and neutrophil counts (2.3 × 10³/L) (Figure, C). During the next 20 months of follow-up, the medications were gradually tapered to tofacitinib, 5 mg, daily (Table), and no flares or adverse effects (eg, infection, cytopenia, or worse osteoporosis) occurred.