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Hidradenitis Suppurativa and Down Syndrome: Systematic Review and Meta-Analysis

Introduction: Hidradenitis suppurativa (HS) is a chronic, inflammatory skin disease, characterized by the presence of painful lesions and recurrent abscesses, and nodules. These lesions can later rupture to form sinus tracts and fistulas. The exact etiology of HS is currently unknown, however a link between genetic, social and environmental factors has been postulated in recent studies. A number of studies have shown a significant association between HS and down syndrome (DS), however the relationship between HS and DS is unclear. We conducted a systematic review and meta-analysis to test for any association between HS and down syndrome.
Methods: A systematic review of existing studies was performed and data was pooled for meta-analysis. Reviews, abstracts and case reports were excluded. Eligible studies were those which investigated HS in cases of down syndrome. Studies that reported DS cases amongst HS patients were not included in the present study.
Results: A total of 6 studies were identified from systematic database searches after applying inclusion and exclusion criteria. Pooled meta-analysis demonstrated a significant association between HS and down syndrome. The pooled proportion of HS cases in the DS group was 10.9% (95%CI,3.8%-27.6%). The pooled proportion of HS cases in the control group was 0.4% (95% CI, 0.2%-0.8%). This difference was significantly different (P<0.001). The odds ratio is 12.02(95%CI,10.91-13.23).
Conclusion: The evidence for hidradenitis suppurativa being associated with down syndrome is very limited. The data is promising however, and further prospective studies with larger cohorts are required to reaffirm the findings in the present review. 