# Upadacitinib successfully treats Hailey-Hailey disease: a cases report and literature review

**Authors:** Zhuochen Wu, Qing Zhu, Xu Yang, Xiaoyu Xie, Guoqiang Zhang

PMC · DOI: 10.3389/fimmu.2025.1676459 · Frontiers in Immunology · 2026-01-14

## TL;DR

A 38-year-old man with Hailey-Hailey disease showed significant improvement after treatment with upadacitinib, a JAK inhibitor, suggesting it could be a new treatment option.

## Contribution

This is the first reported case of upadacitinib successfully treating refractory Hailey-Hailey disease.

## Key findings

- The patient's rash improved significantly within 3 weeks of upadacitinib treatment.
- Improvement continued for 12 weeks with no serious adverse effects.
- Upadacitinib may be an effective and safe treatment for refractory Hailey-Hailey disease.

## Abstract

Hailey-Hailey disease (HHD) is a rare autosomal dominantly inherited skin disorder first described by brothers Howard and Hugh Hailey in 1939. This article reports a case of refractory Hailey-Hailey disease, which was treated with upadacitinib with remarkable efficacy after the unsatisfactory results of traditional treatment regimens. The patient was a 38-year-old male with a 2-year history of the disease. The rash improved significantly at 3 weeks of oral treatment with upadacitinib 15 mg/day and continued to improve at 12 weeks of follow-up without serious adverse effects. Together with the literature review, this study aims to investigate the efficacy and safety of the JAK inhibitor upadacitinib as a novel therapeutic regimen for Hailey-Hailey disease, and to provide a reference for the clinical treatment of refractory cases.

## Linked entities

- **Chemicals:** upadacitinib (PubChem CID 58557659)
- **Diseases:** Hailey-Hailey disease (MONDO:0008218)

## Full-text entities

- **Diseases:** HHD (MESH:D016506), rash (MESH:D005076), autosomal dominantly inherited skin disorder (MESH:D030342)
- **Chemicals:** Upadacitinib (MESH:C000613732)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

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## References

26 references — full list in the complete paper: https://tomesphere.com/paper/PMC12847405/full.md

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Source: https://tomesphere.com/paper/PMC12847405