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Hidradenitis Suppurativa (Advertisement) Home | Specialties | Resource Centers | CME | PDA | Contributor Recruitment April 23, 2006 Articles Images CME Advanced Search Consumer Health Link to this site You are in: eMedicine Specialties > Dermatology > Diseases Of The Adnexa Quick Find Author Information Introduction Rate this Article Hidradenitis Suppurativa Clinical Email to a Colleague Differentials Workup Last Updated: March 23, 2006 Get CME/CE for article Treatment Medication Synonyms and related keywords: HS, acne inversa, acne triad, acne tetrad, hidradenitis Follow-up axillaris, apocrinitis, intertriginous acne, pyoderma fistulans sinifica, Verneuil's disease, Miscellaneous Pictures Verneuil disease, dissecting cellulitis of scalp and neck, acne conglobata, follicular occlusion Bibliography triad, follicular occlusion tetrad, pilonidal sinus, acneiform disorder, apocrine occlusion Click for related AUTHOR INFORMATION Section 1 of 11 images. Author Information Introduction Clinical Differentials Workup Treatment Medication Follow-up Miscellaneous Pictures Bibliography Related Articles Author: Marina Jovanovic, MD, PhD, Chief of Dermatology Ward and Contact Dermatitis Investigative Unit, Clinic Actinomycosis of Dermatoveneroleogic Diseases, Clinical Center, Novi Sad, Serbia and Montenegro; Associate Professor in Dermatology, Medical Faculty, University of Novi Sad, Vojvodina,Serbia and Montenegro Catscratch Coauthor(s): George Kihiczak, MD, Clinical Associate Professor, Department of Dermatology, New Jersey Disease Medical School University Hospital; Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical Erysipelas School Granuloma Inguinale Editor(s): Daniel Siegel, MD, MS, Director, Procedural Dermatology Fellowship Program, Clinical Professor of Dermatology, Department of Dermatology, State University of New York Downstate; David F Butler, MD, (Donovanosis) Professor, Texas A&M University College of Medicine; Director, Division of Dermatology, Scott and White Clinic; Jeff Miller, MD, Assistant Professor, Department of Dermatology, Penn State University, Milton S Hershey Lymphogranuloma Medical Center; Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University; Venereum and Dirk M Elston, MD, Teaching Faculty, Department of Dermatology, Geisinger Medical Center Disclosure Continuing Education CME available for INTRODUCTION Section 2 of 11 this topic. Click Author Information Introduction Clinical Differentials Workup Treatment Medication Follow-up Miscellaneous Pictures Bibliography here to take this CME. Background: Hidradenitis suppurativa (HS) is a disorder of the terminal follicular epithelium in the apocrine gland–bearing skin. HS is characterized by comedolike follicular occlusion, chronic relapsing inflammation, mucopurulent discharge, and progressive scarring. Patient Education Skin, Hair, and Pathophysiology: HS has traditionally been considered a disorder of the apocrine glands. HS was first described Nails Center as a distinct entity in 1839, when Velpeau reported a patient with the superficial abscess formation in the axillary, mammary, and perianal regions. In 1854, Verneuil associated the suppurative process with the sweat glands, and the condition was given its current name. For many years, the condition was described as Verneuil disease, but it Boils Overview subsequently became known as HS. Verneuil did not perform any histopathologic studies, and he conceded that his conclusions were based purely on the characteristic distribution of the condition. Causes of Boils In 1922, Schiefferdecker classified the sweat glands as eccrine and apocrine, and he subsequently localized HS to Symptoms of Boils the apocrine glands. In 1939, Brunsting provided a detailed description of the histologic features of HS. He observed the primary cellular reaction in the lumen of the apocrine glands and in the neighboring periglandular Boils Treatment Boils Treatment connective tissue. Detailing the clinical features of the disease, Brunsting highlighted its frequent association with acne. He noted that HS, dissecting cellulitis of the scalp and neck, and acne conglobata commonly occur in the same patient. He thought that the central pathogenetic event in all 3 conditions was a tendency for follicular hyperkeratinization with secondary bacterial infection. In 1956, Pillsbury et al combined acne conglobata, HS, and dissecting cellulitis under the term follicular occlusion triad. The only flaw in their concept was the focus on apocrine sweat gland involvement. In 1975, Plewig and Kligman added pilonidal sinus as another component to the ensemble, and they introduced the term acne tetrad. Plewig and Kligman pointed out that HS is a misnomer because of the lack of apocrine gland involvement, but they did not present a detailed explanation. In 1989, Plewig and Steger suggested the term acne inversa as an inclusive and accurate name for what was previously called the follicular occlusion triad, or follicular occlusion tetrad. Eventually, HS was accepted as an acneiform disorder that begins with follicular occlusion rather than an infection of the sweat glands. Because HS is actually a defect of follicular epithelium, some authorities have suggested excluding all outdated synonyms for this disease, including HS, and substituting the term acne inversa. The term acne inversa links the pathogenesis to acne and reflects the fact that the condition is an expression of follicular occlusion in localizations inverse to acne vulgaris. 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, although 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 making social contact. In an assessment of socioeconomic effects, Jemec et al noted that the most common problem in both sexes was soreness and that the average patient lost 2.7 days of work per year. In comparison, in Denmark, the average number of work days lost for all reasons was 7.5 days per year per employed person, whereas patients with eczema used 4 weeks of sick leave per year because of their disease. Although HS entails less morbidity than perhaps hand eczema, with regard to its prevalence, it significantly contributes to the average number of work days lost (Danish National Statistical Office, Copenhagen, Denmark, Jemec GB, written communication, 1996). Jemec et al also found sex differences in morbidity. Women lost significantly more days of work (mean, 2.9 d) than men (mean, 1.7 d); this finding suggests that women have either a more fulminant course or other contributing factors. On the other hand, male patients experienced less delay in seeking care from a specialist or hospital; this finding could also suggest a more fulminant course or the presence of confounding factors. The general self-reported level of health, which is well correlated with more objective parameters of morbidity, is significantly worse among HS patients than among healthy control subjects. The mean Dermatology Life Quality Index score for HS is higher than for previously studied skin diseases and correlated with disease intensity as expressed by lesions per month. This suggests that the Dermatology Life Quality Index score may be a relevant outcome measure in future therapeutic trials in HS patients. Race: Although many authors report no racial predilection, an increased frequency is observed in blacks, possibly because blacks have a greater density of apocrine glands than 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. Active genitofemoral lesions occur significantly more often in female patients, whereas perianal involvement tends to be more common in men. No sex difference is seen in the axillary lesions. Large multiheaded comedones are characteristic findings of the disease. Comedones have been suggested as precursor lesions for HS. They appear to be equally distributed in both sexes and sites. Age: The onset of HS peaks in individuals aged 11-50 years, with an average patient age of 23 years. In less than 2% of patients, the disease appears before age 11 years. In extremely rare cases, HS occurs before puberty or after menopause. CLINICAL Section 3 of 11 Author Information Introduction Clinical Differentials Workup Treatment Medication Follow-up Miscellaneous Pictures Bibliography History: HS usually occurs in otherwise healthy individuals, and it rarely begins before puberty. The disease onset is insidious, with early symptoms of pruritus, erythema, and local hyperhidrosis. Later, the lesions become painful. Arthropathy associated with HS may manifest with variable clinical features, ranging from asymmetric pauciarticular
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