Evidence map›Paper›PMID 40661732›Full record

ArticleHealth science reports2025

Hailey-Hailey Disease: An Updated Review With a Focus on Therapeutic Mechanisms.

Mahesh Mathur, Sumit Paudel, Nabita Bhattarai, Sambidha Karki, Sandhya Regmi

Abstract read
In one paragraph

Article in Health science reports, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.

0numbers the graph read from it
0cells of the map it votes in
4citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

4 citing papers in PubMed.

  1. Article
  2. Article
  3. Review
  4. Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

5 authors.

Mahesh MathurDepartment of Dermatology College of Medical Sciences Teaching Hospital Bharatpur Nepal.ORCID https://orcid.org/0000-0001-7831-8949
Sumit PaudelDepartment of Dermatology College of Medical Sciences Teaching Hospital Bharatpur Nepal.
Nabita BhattaraiDepartment of Dermatology College of Medical Sciences Teaching Hospital Bharatpur Nepal.ORCID https://orcid.org/0009-0009-2859-0376
Sambidha KarkiDepartment of Dermatology College of Medical Sciences Teaching Hospital Bharatpur Nepal.ORCID https://orcid.org/0009-0003-6438-1930
Sandhya RegmiDepartment of Dermatology College of Medical Sciences Teaching Hospital Bharatpur Nepal.ORCID https://orcid.org/0009-0005-5844-8910

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Background: Hailey-Hailey disease (HHD), or familial benign chronic pemphigus, is a rare autosomal dominant genodermatosis characterized by chronic, painful, erythematous, erosive plaques and fissures at sites of friction such as the neck, axilla, groin, and perineum. The pathogenesis is due to a mutation in the ATP2C1 gene, which encodes the human secretory-pathway calcium/manganese-ATPase isoform 1 (hSPCA1) that regulates calcium and manganese concentration in the Golgi apparatus. The diagnosis relies on clinical presentation and characteristic histopathological features, notably a "dilapidated brick wall" appearance. There is no cure for this genodermatosis and main aim of management in HHD is to control symptoms and reduce recurrence. Multitude of topical, systemic agents, procedural therapy such as laser therapy and surgery for the treatment of HHD have been reported in the literature. Aims: This comprehensive review aims to discuss the efficacy of current HHD treatments with special focus on therapeutic mechanisms. Conclusion: This review highlights clinical and histological features of HHD and offers guidance for dermatologists involved in managing this distinct dermatosis.

Indexed as

ATP2C1 mutationfamilial benign chronic pemphigusgeneral dermatologygenodermatosisHailey‐Hailey disease

Identifiers

PMID40661732
PMCPMC12257355

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.