Clinical Features of Hidradenitis Suppurativa in Down Syndrome Patients: A Multicenter Spanish Study

Abstract

Background:

Current evidence indicates a significant association between hidradenitis suppurativa (HS) and Down syndrome (DS), though the underlying mechanisms remain unclear.

Objective:

To describe the demographic profile and clinical manifestations of HS in patients with DS, including phenotype, disease severity, comorbidities, and therapeutic approaches.

Methods:

This was an observational, cross-sectional, retrospective, multicenter study including consecutive patients with both DS and HS across 15 Spanish hospitals. Primary endpoints were HS clinical phenotype and severity; secondary endpoints included comorbidities and therapeutic burden.

Results:

Sixty-nine patients with DS and HS were included. Phenotypic data were available for 68 patients (98.6%), with 38.2% classified as inflammatory, 30.9% as follicular, and 30.9% as mixed phenotype. The median (interquartile range) age at disease onset was 15.0 years (13.0-18.3), and the age at diagnosis was 20.0 years (16.0-30.0). Most patients (50.7%) had Hurley stage I disease. The most affected sites were the groin (69.6%) and axilla (63.8%). The inflammatory phenotype group showed a significantly higher prevalence of systemic hypertension, dyslipidemia, endocrinological disorders (particularly hypothyroidism), and cardiovascular disease compared to the other phenotypes (P = .0475, .0493, and .0042, respectively). Treatment strategies did not differ significantly across phenotypes.

Conclusions:

Patients with both DS and HS had an earlier disease onset than the general HS population. Most patients were stage I, with axillae and groin affected. The inflammatory phenotype was associated with an increased prevalence of systemic hypertension, dyslipidemia, endocrine comorbidities (especially hypothyroidism), and cardiovascular disease. Finally, this study suggested that DS did not contraindicate or restrict the use of HS treatments.