What is Hidradenitis Suppurativa?!
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Background
This is a chronic and relapsing condition occurring within apocrine sweat glands, most commonly in women. Deep seated inflammatory nodules form in the axilla, groin, pubic region, and perineum. It is an androgen-dependent disorder and results from keratinous material occluding the sweat pores. There is an association with lithium therapy and Crohn's disease.
Etiology
HS often occurs in multiple members within families, suggesting a genetic predisposition (5). Various etiological agents have been associated. The increased incidence of HS in obese women with acne led to the theory of HS being associated with an hyperandrogenic endocrine disorder (6,7). One study did not find evidence for biochemical hyperandrogenism in women affected with HS (8). Thus, the role of androgens in HS is still not clear. Smoking (9), lithium (10) and oral contraceptives (11) may also be associated with HS, possibly as triggering factors. Nonetheless, the etiology of the disease is unknown.
Frequency
In the US: In the United States, the prevalence of HS appears to be 1-2% in the general population.
Internationally: The prevalence of HS appears to be 1% of the general population; it was 4% in a group of young adults who were treated at a clinic for sexually transmitted diseases. HS is probably more common than once thought, but the diagnosis is frequently ignored or missed. HS has a worldwide distribution, though hot, humid environments tend to support its development.
Mortality/Morbidity
The death rate is similar to that in the general adult population.
Although rare complications of the disease are described, little is known about the typical effects of HS. HS is a chronic disabling disorder that relentlessly progresses and leads to keloids, contractures, and immobility; patients can become outcasts or at least have difficulty in making social contact.
Pathogenesis
Historically, apocrinitis was believed to be the defining histologic feature and pathogenetic mechanism of HS (12,13). Characteristic lobular abscesses in the apocrine gland, demonstrated histologically, seemed to further implicate apocrine gland involvement in the manifestation of disease (12-14). Recently, histological evidence points to HS being a follicular disease (1,15-19). Histological examination in the majority of specimens reveals follicular involvement, including poral occlusion and folliculitis (1). Apocrinitis as the dominant histological feature is found in only a small number of specimens (1,17). Furthermore, a paucity of apocrine glands was demonstrated in the genitofemoral region, one area commonly affected by HS (1). This finding supports the theory that apocrine gland inflammation is not the etiological and pathogenetic mechanism of HS, but rather a secondary manifestation of follicular involvement (1).
Clinical Features
HS may arise singularly or multifocally in the genitofemoral, perianal and axillary areas (1-4). The lesions are painful and have a foul odor attributed to bacterial colonization. Erythematous dermal abscesses form that measure up to 2 cm in diameter. Untreated abscesses will gradually increase in size and may drain to the surface. The course of HS is chronic and remitting, with new abscesses arising in previously unaffected areas or in regions of past involvement. Scarring, fibrosis and sinus tract formations are manifestations of late disease. Strictures can occur secondary to sinus tracts; fistulas may also complicate HS. Squamous cell carcinoma is a rare sequella of longstanding HS (20-24). These cancers may be locally aggressive with distant metastases and a high mortality rate (25).
Race/Sex/Age
Race: Although many authors report no racial predilection, an increased incidence is observed in blacks, possibly because blacks have a greater density of apocrine glands compared with whites.
Sex: Although HS is widely considered to occur more frequently in females than in males, with a ratio as high as 2-5:1, reports on sex prevalence are controversial. Axillary involvement is probably more common in women than in men, whereas perianal and anogenital involvement tends to be more common in men.
Age: The onset of HS peaks in individuals aged 11-50 years, with an average patient age of 23 years. In fewer than 2% of patients, the disease appears before they are aged 11 years.
In extremely rare cases, HS occurs before puberty or after menopause.
Therapy
Treatment of hidradenitis suppurativa is challenging. Late stage disease, evidenced by the formation of sinus tracts, fibrosis and scarring, usually necessitates surgical intervention. Early HS is often best treated with antibiotics in our experience, although few clinical trials are available (39). Three months' of treatment with topical clindamycin decreased the number of abscesses, inflammatory nodules and pustules in twenty-seven patients with chronic HS (40). Systemic tetracycline therapy has shown similar clinical effectiveness (39). Others and also we often recommend intrealesional corticosteroids in early stage disease (41). The use of cyproterone acetate and ethinyl estradiol achieved successful clinical results in four women with chronic HS (42); clinical improvement with cyproterone acetate and ethinyl estradiol was also described in another study (43). Isotretinoin is only slightly effective in controlling the disease; clinical improvement is seen in patients with mild HS (44). The clearing of chronic, refractory perianal HS was seen after treatment with cyclosporin for concomitant pyoderma gangrenosum (38).
Medical therapy is of limited value once HS has progressed past its early stage (2). The surgical option of choice for late stage HS is wide local excision with healing by secondary intention (41). One study examined patients with chronic HS who had undergone surgery between the years of 1976 and 1997 (4). An estimated 72-month follow-up revealed that 45% of the patients had recurrence of local HP (4). A 100% recurrence rate was reported after drainage procedures, while limited and wide local excision techniques had a recurrence rate of 42.8% and 27%, respectively (4). More recently, carbon dioxide laser excision has been proposed as a better alternative to conventional surgery (2,45). Carbon dioxide laser excision offers better hemostasis and visualization of abscessed tissue than conventional surgical techniques, allowing more accurate excision (2).
References
J Am Acad Dermatol 1996 Aug;35(2 Pt 1):191-4
Int J Colorectal Dis 1993 Sep;8(3):117-9
Inflamm Bowel Dis 2001 Nov;7(4):323-6
Br J Dermatol 1996 Jun;134(6):1057-9
Am J Med Sci 2001 Mar;321(3):195-7
Br J Dermatol 1995 Aug;133(2):254-8
Acta Derm Venereol 2001 May;81(2):96-9
J Eur Acad Dermatol Venereol 2000 Sep;14(5):389-92
Gynecol Oncol 1999 Nov;75(2):285-8
J Am Acad Dermatol 1998 Dec;39(6):971-4
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