Autoimmune diseases

Baracitinib effective in refractory autoimmune blistering disease

The JAK-1/2 inhibitor baricitinib has been effective in a single case report of a patient with long-standing lichen planus pemphigoides (LPP).

LPP is a rare but distinct clinical entity characterised by features of both lichen planus (LP) and bullous pemphigoid (BP).

The case report from Sinclair Dermatology in Melbourne described a 36-year old male with a 17-year history of “recalcitrant, pruritic and excoriated plaques associated with blisters on his abdomen and limbs”.

A diagnosis of LPP had previously been confirmed and the patient unsuccessfully treated with multiple agents including topical and oral corticosteroids, methotrexate, azathioprine, ciclosporin, doxycycline and psoralen and ultraviolet A therapy.

“Baricitinib was considered given the shared features between LPP and LP, and previous success treating LP variants with JAK-1/2 inhibition at our centre.”

The patient was started on 3.4 mg baracitinib twice daily with a significant improvement in pruritis reported after two weeks of treatment.

“Four weeks after commencing treatment, a further reduction of LPP lesions was noted, and the treatment was tapered. At 6 months, near complete resolution of LPP was sustained on a daily dose of baricitinib of 3.4 mg.”

The case report, published as Correspondence in the journal Clinical and Experimental Dermatology, said it was plausible that baricitinib suppresses the lichenoid tissue reaction in LPP.

“As lichenoid skin changes precede the formation of blisters in most cases of LPP, exposure to various autoantigens secondary to degeneration of the basal epidermis is thought to be a triggering event.”

“To our knowledge, this is the first case of LPP successfully treated with baricitinib. While further studies are required to support our findings, in the absence of effective treatments for recalcitrant LPP, baricitinib may be a suitable addition to the therapeutic armamentarium,” the authors concluded.

Disclosure: Professor Rod Sinclair is on the pharmaceutical advisory board of Eli Lilly.

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