Abstract | Hidradenitis suppurativa (HS) kronična je, recidivirajuća, često iscrpljujuća upalna bolest pilosebacealnog folikula koja se obično prezentira nakon puberteta pojavom dubokih, bolnih, upalnih nodusa i apscesa na intertriginoznim predjelima. Suprotno akni, HS najčešće zahvaća područja aksilarne, ingvinalne, anogenitalne i inframamarne regije, pa se stoga ponekad za bolest koristi izraz acne inversa. Prevalencija HS u Europi procijenjena je na oko 1%, a žene su zahvaćene otprilike tri puta češće nego muškaraci. Vrhunac incidencije je u drugom i trećem desetljeću života. Bolest se povezuje s brojnim komorbiditetima, uključujući sindrome folikularne okluzije, upalne bolesti crijeva, metabolički sindrom, artritis i depresiju. Hidradenitis suppurativa se također može pojaviti u sklopu autoinflamatornih sindroma poput PASH, PAPASH, PsAPASH i PASS sindroma. Bolest je prvenstveno uzrokovana folikularnom okluzijom, uz sekundarno zahvaćanje apokrinih žlijezda. Etiologija nije do kraja razjašnjena, no uključuje poremećaj urođene imunosti, kao i rizične čimbenike povezane s bolešću, poput pušenja, pretilosti i genetskih čimbenika. Klinički, karakteristične kožne lezije su supkutani čvorovi, duboki dermalni apscesi, dvostruki komedoni i sinusni kanali. Kako bolest napreduje, razvija se fibroza, induracija kože i dermalne kontrakture. Dijagnoza se postavlja klinički, na temelju karakterističnih kožnih lezija. Hurleyev sustav klasifikacije HS pomaže u donošenju odluka o terapiji. Sukladno europskim smjernicama, lokalno recidivirajuće lezije mogu se tretirati klasičnim kirurškim postupcima (ekscizija) ili primjenom lasera. Široko rasprostranjene lezije zahtijevaju farmakoterapiju, bilo kao monoterapiju ili u kombinaciji s radikalnim kirurškim zahvatima. Farmakoterapija uključuje lokalne ili sustavne antibiotike (tetracikline te klindamicin i rifampicin), retinoide (acitretin) te biološke lijekove (adalimumab, infliksimab). Kako bi se spriječile teške posljedice bolesti i poboljšala kvaliteta života bolesnika, rana dijagnoza i liječenje HS od iznimne su važnosti. |
Abstract (english) | Hidradenitis suppurativa (HS) is a chronic, recurrent, often debilitating inflammatory disease of the pilosebaceous follicle, typically appearing after puberty with deep, painful, inflammatory nodules and abscesses in the intertriginous areas. Contrary to acne, it occurs most commonly in the axillary, inguinal, anogenital and inframammary regions, and is sometimes referred to as acne inversa. The prevalence of HS in Europe has been estimated to be approximately 1%, with women being affected about three times more often than men. The condition has its peak incidence in the second and third decades of life. The disease has been associated with multiple comorbidities, including follicular occlusion syndromes, inflammatory bowel disease, metabolic syndrome, arthritis and depression. Hidradenitis may also appear in association with autoinflammatory syndromes, like the PASH, PAPASH, PsAPASH and PASS syndroms. The disease is caused primarily by follicular occlusion, with secondary involvement of the apocrine glands. The etiology is still not fully elucidated; however, it involves disrupted innate immune response and disease-associated risk factors such as smoking, obesity and genetic mutations. Clinically, characteristic skin lesions are subcutaneous nodules, deep dermal abcesses, double comedones and sinus tracts. As the disease process continues, fibrosis, induration of the skin and dermal contractures occur. Diagnosis is made clinically, based on the characteristic skin lesions. The Hurley staging system of HS helps faciliate therapeutic decision. According to the European guidelines, locally recurrent lesions can be treated with classical surgery (excision) or laser techniques. However, widely spread lesions require drug treatment, either as monotherapy or in combination with radical surgery. Drug therapies include topical or systemic antibiotics (tetracyclines or clindamycin and rifampicin), retinoids (acitretin) and biologics (adalimumab and infliksimab). In order to prevent the devastating consequences of the disease and improve the quality of life in patients, early diagnosis and treatment of HS are extremely important. |