Evidence map›Paper›PMID 40754446›Full record

ReviewEndocrine journal2025

A case of Hailey-Hailey disease accompanied by Cushing's syndrome and adrenal insufficiency due to long-term usage of topical steroids with review of literature.

Yuichiro Iwamoto, Masahiro Komi, Yoshiro Fushimi, Tomohiko Kimura, Masashi Shimoda, Shuhei Nakanishi, Yumi Aoyama, Tomoatsu Mune, Hideaki Kaneto

Abstract readCase ReportsReview
In one paragraph

Review in Endocrine journal, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing 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

1 citing paper in PubMed.

  1. Article
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

9 authors.

Yuichiro IwamotoDepartment of Diabetes, Endocrinology and Metabolism, Kawasaki Medical School, Okayama 701-0192, Japan.ORCID http://orcid.org/0000-0001-8162-359X
Masahiro KomiDepartment of Diabetes, Endocrinology and Metabolism, Kawasaki Medical School, Okayama 701-0192, Japan.
Yoshiro FushimiDepartment of Diabetes, Endocrinology and Metabolism, Kawasaki Medical School, Okayama 701-0192, Japan.ORCID http://orcid.org/0000-0002-9618-8698
Tomohiko KimuraDepartment of Diabetes, Endocrinology and Metabolism, Kawasaki Medical School, Okayama 701-0192, Japan.ORCID http://orcid.org/0000-0003-3986-9494
Masashi ShimodaDepartment of Diabetes, Endocrinology and Metabolism, Kawasaki Medical School, Okayama 701-0192, Japan.ORCID http://orcid.org/0000-0002-4223-9613
Shuhei NakanishiDepartment of Diabetes, Endocrinology and Metabolism, Kawasaki Medical School, Okayama 701-0192, Japan.ORCID http://orcid.org/0000-0003-2640-9632
Yumi AoyamaDepartment of Dermatology, Kawasaki Medical School, Okayama 701-0192, Japan.ORCID http://orcid.org/0000-0003-3701-5575
Tomoatsu MuneDepartment of Diabetes, Endocrinology and Metabolism, Kawasaki Medical School, Okayama 701-0192, Japan.
Hideaki KanetoDepartment of Diabetes, Endocrinology and Metabolism, Kawasaki Medical School, Okayama 701-0192, Japan.ORCID http://orcid.org/0000-0001-7898-1943

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Hailey-Hailey disease (HHD), or familial benign chronic pemphigus, is a rare autosomal dominant disorder characterized by recurrent vesicles and erosions in intertriginous areas. Topical corticosteroids are the primary treatment, but their potential systemic side effects are often overlooked. Prolonged use on compromised skin can lead to excessive absorption, increasing the risk of iatrogenic Cushing's syndrome and adrenal insufficiency. Here, we report the case of a 50-year-old woman with HHD who had been using topical clobetasol or betamethasone for over 10 years, reaching doses up to 50 g/day. She developed Cushingoid features, metabolic abnormalities, and suppression of the hypothalamic-pituitary-adrenal (HPA) axis. After tapering off topical corticosteroids, she developed adrenal insufficiency and associated withdrawal symptoms. Following the initiation of hydrocortisone replacement therapy, psychiatric symptoms, impaired glucose tolerance, and osteoporotic fractures emerged, suggesting exacerbation of iatrogenic Cushing's syndrome. This case highlights the risk of systemic complications from chronic topical corticosteroid use, particularly in high-absorption areas. Gradual dose reduction, close endocrine monitoring, and individualized tapering strategies are essential to prevent severe outcomes. Clinicians should be aware of potential adrenal suppression and consider endocrine evaluation in patients receiving prolonged, high-dose topical corticosteroid therapy.

Indexed as

Adrenal InsufficiencyBetamethasoneClobetasolCushing SyndromeGlucocorticoidsPemphigus, Benign FamilialAdministration, TopicalFemaleHumansHydrocortisoneMiddle AgedBetamethasoneClobetasolGlucocorticoidsHydrocortisoneAdrenal insufficiencyCushing’s syndromeFamilial chronic benign pemphigusSteroidSteroid withdrawal symptom

Identifiers

PMID40754446
PMCPMC12702672

What OpenQuestion holds

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LicenceCC BY-NC-ND
Read underepoch 390

Registered trials

None linked

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.