Family History Plays Significant Role in Severity of Hidradenitis Suppurativa

Individuals living with hidradenitis suppurativa (HS) who have a family history of inflammatory conditions are more likely to present with Hurley stage 2 or 3 versus stage 1, new findings suggest, highlighting the value of evaluating family history in those living with HS.1

These findings connecting the shared inflammatory pathways between various autoinflammatory conditions and HS resulted from a recent retrospective analysis. Defne Özkoca, from the Koç University School of Medicine Department of Dermatology and Venerology in Istanbul, led a team of investigators in this study.

“In this retrospective study, we analyzed 248 HS patients admitted to Koç University Hospital between January 2019 and July 2024,” Özkoca and colleagues wrote.1 “Routine clinical assessments included detailed medical and family histories, specifically concerning Behçet's disease, psoriasis, IBD, and rheumatologic diseases.”

Some of these diseases had previously been shown to be linked to HS.2 The investigative team looked at 248 individuals for the purposes of their retrospective study, with each of these subjects having been admitted to Koç University Hospital between January 2019 - July 2024 and each subject living with HS.

The team would determine participants' severity level in their HS through the use of the Hurley staging system for classification. They included comprehensive histories of medical needs and family background, placing their focus on psoriasis, Behçet’s disease, inflammatory bowel disease (IBD), and rheumatologic conditions, in their routine clinical evaluations of subjects.

The investigators conducted the necessary statistical analyses through the use of SPSS version 21, employing chi-square and Fisher’s exact tests. Given the need for comparisons in their analysis, the team created a control group of 70 age- and gender-matched subjects without HS. A P-value of < .05 was decided by the investigators to be statistically significant.

Among the 248 HS patients, the mean age at disease onset was 28.4 years, and 60.1% of the cohort was male. The majority were categorized as Hurley stage 1 (42.7%) or stage 2 (39.9%), while 17.3% were classified as stage 3.

Higher rates than those of the control arm were identified by the research team among those with HS for arthralgia, metabolic syndrome, ankylosing spondylitis, and follicular occlusion tetrad. The team additionally found that a family history of HS, Behçet’s disease (P = .023), and rheumatologic conditions (P < .01) were shown to have been more frequently seen among those in the HS arm of the analysis.

A familial history of inflammatory diseases among those presenting with Hurley stage 2 or 3 was identified as more likely, rather than stage 1 (P = .047). Overall, the investigators' results highlight the notable and impactful role of family history in severity of HS.

Recent analyses the investigative team highlighted in addition had pointed to mutations in the MEFV gene, which encodes pyrin, as having been detected in those with HS.3 Such data could indicate overlapping inflammatory pathways with other autoinflammatory conditions, and the team noted that such a genetic connection would support the involvement of key inflammatory mechanisms. These mechanisms could include interleukin (IL)-1 and TNF-α signaling.

In their description of the study's primary limitations, the investigators noted that its retrospective design and single-center scope would be examples. Despite this acknowledgement, they added that their research provide some valuable insights into the link between HS severity and inflammatory conditions with a strong familial component.

The team also highlighted prior findings linked vitamin D levels to HS severity, as vitamin D deficiency was shown to be more prevalent among individuals with HS who did not demonstrate any comorbid inflammatory conditions (P = .028). Despite such conclusions, additional research with larger cohorts may be required to clarify the clinical significance of vitamin D deficiency in those with HS.

The accumulation of data identified in the investigators' analysis suggest that early identification and management of familial risk factors could aid in the optimizing of HS management, as could the shared genetic framework between HS and other inflammatory diseases.

“Further multicenter prospective studies are needed to clarify the underlying molecular mechanisms linking familial inflammatory disease history and HS disease course, potentially leading to more targeted therapeutic approaches,” they concluded.1

References

1. Özkoca, D., Özcanlı, A., Er Gülbezer, E., Salıcı, N.S., Yaylı, S. and Vural, S. (2025), Association Between Familial Inflammatory Diseases and Hidradenitis Suppurativa Severity: A Single-Center Study. Int J Dermatol. https://doi.org/10.1111/ijd.17747.

2. T. V. Nguyen, G. Damiani, L. A. V. Orenstein, I. Hamzavi, and G. B. Jemec, “Hidradenitis Suppurativa: An Update on Epidemiology, Phenotypes, Diagnosis, Pathogenesis, Comorbidities and Quality of Life,” Journal of the European Academy of Dermatology and Venereology 35, no. 1 (2021): 50–61.

3. J. W. Frew, “Unravelling the Complex Pathogenesis of Hidradenitis Suppurativa,” British Journal of Dermatology 192, no. Supplement_1 (2025): i3–i14.