Hidradenitis suppurativa in autoinflammatory syndromes

Hidradenitis suppurativa (HS) is a chronic, recurrent inflammatory skin disease characterized by deep-seated, painful inflammatory lesions. HS can present as a solitary condition or a syndromic disease associated with diagnostic delays and treatment challenges. The underlying pathophysiology of autoinflammatory syndromes is a combination of genetic mutations in autoinflammatory genes and molecular mechanisms that drive autoinflammation, with a complex interplay between the innate and adaptive immune systems. Clinical manifestations include a combination of skin, musculoskeletal, and gastrointestinal signs and symptoms frequently appearing in a temporal order described as an "autoinflammatory march." Management is complex and centers around systemic antimicrobials and biologics. Future research should further elucidate the triggers and drivers of autoinflammation to develop targeted and personalized treatment approaches. The role of dermatologists in autoinflammatory syndromes is essential in recognizing patients at risk for syndromic HS to provide adequate and timely treatment.