Rapid and Sustained Improvement of Lichen Planus Pemphigoides With the JAK1 Inhibitor Upadacitinib

ABSTRACT Lichen planus pemphigoides (LPP) is a rare autoimmune blistering disorder characteris ed by lichenoid plaques and blister formation. Current treatments are often ineffective, and there is a lack of standardised guidelines. The disease's pathogenesis involves both T‐cell‐mediated lichenoid dermatitis and autoantibody formation, presenting treatment challenges, especially in refractory cases. While previous therapies have focused on immunosuppressive agents, many patients fail to achieve sustained remission. Here, we report a case of LPP successfully treated with the Janus kinase 1 (JAK1) inhibitor upadacitinib. The patient, a 56‐year‐old female with pruritic lichenoid lesions and blisters, experienced rapid symptom resolution following treatment. Notably, pruritus subsided within days, and both skin lesions and anti‐BP180 antibody levels improved substantially within 1 month. Remarkably, the patient remained nearly symptom‐free for 6 months posttreatment. This case demonstrates that JAK1 inhibition can offer rapid and sustained clinical improvement in LPP, with the potential for disease modification. These findings suggest that early, targeted intervention with JAK inhibitors may provide a new therapeutic avenue for autoimmune skin diseases, particularly those resistant to traditional treatments.

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Publication Details

Journal
JEADV Clinical Practice
Published
2024-12-18
DOI
https://doi.org/10.1002/jvc2.603
Primary Topic
Autoimmune Bullous Skin Diseases
Type
article
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article

Rapid and Sustained Improvement of Lichen Planus Pemphigoides With the JAK1 Inhibitor Upadacitinib

Christoph Schlapbach, Ronald Wolf, Louise Gueissaz
JEADV Clinical Practice
Autoimmune Bullous Skin Diseases
article

Rapid and Sustained Improvement of Lichen Planus Pemphigoides With the JAK1 Inhibitor Upadacitinib

Christoph Schlapbach, Ronald Wolf, Louise Gueissaz
article en

Abstract

ABSTRACT Lichen planus pemphigoides (LPP) is a rare autoimmune blistering disorder characteris ed by lichenoid plaques and blister formation. Current treatments are often ineffective, and there is a lack of standardised guidelines. The disease's pathogenesis involves both T‐cell‐mediated lichenoid dermatitis and autoantibody formation, presenting treatment challenges, especially in refractory cases. While previous therapies have focused on immunosuppressive agents, many patients fail to achieve sustained remission. Here, we report a case of LPP successfully treated with the Janus kinase 1 (JAK1) inhibitor upadacitinib. The patient, a 56‐year‐old female with pruritic lichenoid lesions and blisters, experienced rapid symptom resolution following treatment. Notably, pruritus subsided within days, and both skin lesions and anti‐BP180 antibody levels improved substantially within 1 month. Remarkably, the patient remained nearly symptom‐free for 6 months posttreatment. This case demonstrates that JAK1 inhibition can offer rapid and sustained clinical improvement in LPP, with the potential for disease modification. These findings suggest that early, targeted intervention with JAK inhibitors may provide a new therapeutic avenue for autoimmune skin diseases, particularly those resistant to traditional treatments.

JEADV Clinical PracticeVol. 4(2)
University Hospital of Bern (CH)
Good health and well-being
Openalex Percentile: Top 62%
Autoimmune Bullous Skin Diseases
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