NON RARE IN EUROPE: Hidradenitis suppurativa

Hidradenitis suppurativa (HS), also known as acne inversa or Verneuil disease, is a chronic, recurrent, inflammatory skin disease affecting the hair follicles in areas bearing apocrine glands, primarily the axillae (armpits), groin, perianal region, and inframammary folds (under the breasts). The condition is characterized by painful, deep-seated nodules, abscesses, draining sinus tracts (tunnels), and scarring. Lesions typically begin as firm, tender subcutaneous nodules that may progress to form abscesses that rupture and discharge purulent or serosanguinous fluid. Over time, interconnecting sinus tracts and fibrotic scarring develop, significantly impairing quality of life and causing considerable pain, restricted mobility, and psychosocial distress including depression and social isolation. The pathogenesis of HS involves follicular occlusion followed by rupture of the hair follicle, leading to an intense inflammatory response in the surrounding dermis and subcutaneous tissue. Risk factors include obesity, smoking, and family history, with approximately one-third of patients reporting affected relatives. A familial form has been linked to mutations in gamma-secretase complex genes (NCSTN, PSENEN, PSEN1). The disease is classified using the Hurley staging system into three stages of increasing severity. Treatment is multimodal and stage-dependent: mild disease may be managed with topical antibiotics (such as clindamycin), while moderate-to-severe disease may require systemic antibiotics (tetracyclines, combination clindamycin-rifampicin), hormonal therapies, or biologic agents. Adalimumab, a TNF-alpha inhibitor, is the first biologic approved specifically for moderate-to-severe HS. Secukinumab, an IL-17A inhibitor, has also received approval. Surgical interventions, including incision and drainage, deroofing, and wide local excision, are important components of management, particularly for chronic, scarred lesions and sinus tracts. Despite available treatments, HS remains a challenging condition with no definitive cure, and many patients experience a relapsing course.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

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