Hidradenitis Suppurativa
Section snippets
Definition
HS is defined clinically by its various features and by its chronicity. It is a recurrent disease, classically, but not exclusively, in the apocrine gland–bearing areas of the skin, manifesting as painful, recurrent, deep-seated, inflamed nodules that can result in abscesses, sinuses, and varying degrees of chronic draining sinus tracts with scarring, disfigurement, and disability.
The Second International HS Research Symposium (San Francisco, March 2009) adopted the following consensus
Diagnostic criteria
The Second International HS Research Symposium also adopted the following diagnostic criteria:
- 1.
Typical lesions: either deep-seated painful nodules (blind boils) in early primary lesions or abscesses, draining sinuses, bridged scars, and tombstone open comedones in secondary lesions
- 2.
Typical topography: axillae, groin, genitals, perineal, and perianal region, buttocks, infra- and intermammary folds
- 3.
Chronicity and recurrences.
These 3 criteria must be met to establish the diagnosis.6
HS is recognized
Differential diagnosis
HS has an extensive differential diagnosis (Box 2).8 The appearance, age of onset, typical locations, poor response to antibiotics, and lack of signs of systemic sepsis can all help distinguish this condition, so the diagnosis should be obvious. There are few conditions that cause recurrent abscesses and sinus tract formation in the intertriginous skin areas, such as Crohn disease, ulcerative colitis, and granuloma inguinale. The most common differential diagnoses are the follicular pyodermas:
Prevalence and epidemiology
HS is a common, forgotten, and orphaned disease. It is often not recognized by physicians, even dermatologists, and the results are devastating for patients. HS is mistakenly referred to as a rare disease. The global prevalence has been reported as between 1% and 4%, depending on the definitions used.9 Jemec10 reported a prevalence of 4% in a series of self-reported cases, with a high prevalence in young adults that was confirmed in another study.11 Revuz reported the prevalence in persons 55
Etiology
Several factors are related to the development of HS.
Clinical Description
HS classically occurs in the early 20s. The onset can be insidious, with apparently random small lesions that develop as a red, indurated papule, pustule, or nodule that may resolve without leaving any mark (Box 3). The lesions may develop in only 1 area, or several areas over weeks or months. The discomfort varies from vague itching to mild to moderate pain. In some cases, the onset can be severe, even frightening, with large, deep, painful lesions that result in restriction of activities. Too
Clinical course
The mean age of onset is 22.1 years and HS lasts about 19 years.7 It can remit or partially remit with pregnancy and breastfeeding. The course for patients can be variable. It may be intermittent and benign, with mild but chronic painful disease, acute exacerbations, premenstrual flares, and usually resolution after menopause. It may remit for weeks to months, lesions may flare continuously or only intermittently, and marked involvement may occur in only 1 area with a long deep-draining sinus
Hurley stages
In the recent report by Canoui-Poitrine and colleagues,16 68.2% of patients are Hurley stage I, 27.6% Hurley stage II, and 3.9% Hurley stage III (Box 4). To more accurately assess outcome variables related to HS treatment, the Sartorius score has been developed to monitor patients. It is to act as a complimentary system to the Hurley classification, and is presently undergoing evaluation as a research tool.33
Morbidity/quality of life
It is usual for patients with severe disease to be unemployable and socially isolated because of the painful, draining, and malodorous lesions.34 HS has a profoundly negative effect on patients, not only at a physical level but socially and economically. von der Werth and Jemec35 studied 144 patients with HS with a dermatology life quality index and found a higher morbidity index than in mild to moderate psoriasis or alopecia. The high score was related to pain, malodorous discharge, intimate
Associated diseases
HS has been associated with severe acne (acne conglobata), dissecting cellulitis of the scalp, and pilonidal cysts (Box 5).40, 41 Pilonidal cysts have been associated with HS in up to 30% of cases. Acne vulgaris is only found with HS in about 10% of women and 30% of men but a history of long-lasting, scarring acne was found in 23% of women and 44% of men. Dissecting cellulitis of the scalp is rare (1%); Revuz5 has never seen the follicular triad in 500 patients he has followed.
Keratitis-ichthyosis-deafness Syndrome
Two rare pigmentary disorders have been associated with HS. Dowling-Degos disease is a genetic condition with flexural pigmented macules in the axilla, neck, and groin. Kitamura disease has reticulate acropigmentation on hands and feet. The association of these conditions with HS is soft.9 There are many reports of rheumatologic associations or complications of HS, especially in African American men. This seems to be related to chronic inflammation.42, 43, 44, 45 SAPHO syndrome has been
Complications
HS is associated with several complications, especially in long-term untreated disease (Box 6). Fistulae are rare and associated with long-standing disease.8 The most common complications are anal and perianal fistulae, and it is speculated that these occur because of repeated or persistent abscess formation in the anal glands between the internal and external sphincter.54 Arthropathy can be associated with or can complicate HS (see earlier discussion). In late disease in the genital and
Treatment
There is no single effective treatment or cure for HS. The only permanent cure has been reported for very severe HS (Hurley III) with wide surgery. Most patients with HS require a combination of medical and surgical strategies, beginning with gentle atraumatic care.
General care for HS is based on clinical experience:
- •
Gentle local hygiene. Washing with a mild nonsoap cleansing bar is all that is necessary. If there is odor then an antiseptic cleanser with triclosan could be used. Washing should
The future
Extensive information is available from the Hidradenitis Suppurativa Foundation at www.HS-foundation.org and the International Society for the Study of Vulvovaginal Disease at its educational site www.issvd.org, and the subject is regularly updated at www.UpToDate.com.
Patients with HS (and their physicians, surgeons, and dieticians) need to understand this disease to develop realistic expectations and to work with each other for the best outcome. They need to know that HS is not contagious and
References (98)
- et al.
Hidradenitis suppurativa: a comprehensive review
J Am Acad Dermatol
(2009) - et al.
The prevalence of hidradenitis suppurativa and its potential precursor lesions
J Am Acad Dermatol
(1996) - et al.
Hidradenitis suppurativa in a pre-pubertal female
J Pediatr Adolesc Gynecol
(2009) - et al.
Clinical characteristics of a series of 302 French patients with hidradenitis suppurativa, with an analysis of factors associated with disease severity
J Am Acad Dermatol
(2009) - et al.
Inversa acne (hidradenitis suppurativa): a case report and identification of the locus at chromosome 1p21.1-1q25.3
J Invest Dermatol
(2006) - et al.
Hidradenitis suppurativa: pathogenesis and management
Br J Plast Surg
(2003) - et al.
Lithium therapy associated with hidradenitis suppurativa: case report and a review of the dermatologic side effects of lithium
J Am Acad Dermatol
(1995) - et al.
Functional significance of non-neuronal acetylcholine in skin epithelia
Life Sci
(2007) - et al.
Expanding the roles of antimicrobial peptides in skin: alarming and arming keratinocytes
J Invest Dermatol
(2007) - et al.
Quality of life impairment in hidradenitis suppurativa: a study of 61 cases
J Am Acad Dermatol
(2007)
SAPHO syndrome
Rheum Dis Clin North Am
Ann Dermatol Venereol
Surgical unroofing for hidradenitis suppurativa, why and how?
J Am Acad Dermatol
Long-term results of isotretinoin in the treatment of 68 patients with hidradenitis suppurativa
J Am Acad Dermatol
Topical clindamycin versus systemic tetracycline in the treatment of hidradenitis suppurativa
J Am Acad Dermatol
Infliximab for severe hidradenitis suppurativa: transient clinical efficacy in 7 consecutive patients
J Am Acad Dermatol
A prospective clinical trial of open-label etanercept for the treatment of hidradenitis suppurativa
J Am Acad Dermatol
Etudes sur les tumeurs de la peau; de quelques maladies des grandes sudoripares
Archives Generales de Medicine
Hidradenitis suppurativa: a disease of follicular epithelium, rather than apocrine glands
Br J Dermatol
“Hidradenitis suppurativa” is acne inversa! An appeal to (finally) abandon a misnomer
Int J Dermatol
What’s new in acne inversa (alias hidradenitis suppurativa)?
J Eur Acad Dermatol Venereol
Hidradenitis suppurativa
J Eur Acad Dermatol Venereol
The natural history of hidradenitis suppurativa
J Eur Acad Dermatol Venereol
Epidemiology
The symptomatology of hidradenitis suppurativa in women
Br J Dermatol
Pathogenesis, clinical features and management of hidradenitis suppurativa
Ann R Coll Surg Engl
Hidradenitis suppurativa–characteristics and consequences
Clin Exp Dermatol
Prepubertal hidradenitis suppurativa: two case reports and review of the literature
Pediatr Dermatol
Endocrine factors in pre- and postmenopausal women with hidradenitis suppurativa
Br J Dermatol
Hidradenitis suppurativa in 64 female patients: retrospective study comparing oral antibiotics and antiandrogen therapy
J Cutan Med Surg
Mediation of hidradenitis suppurativa by androgens
Br Med J (Clin Res Ed)
Cutaneous adverse events in renal transplant recipients receiving sirolimus-based therapy
Transplantation
HS patients’ frequently asked questions
Cigarette smoking as a triggering factor of hidradenitis suppurativa
Dermatology
What causes hidradenitis suppurativa?
Exp Dermatol
Lipid raft-enriched stem cell-like keratinocytes in the epidermis, hair follicles and sinus tracts in hidradenitis suppurativa
Exp Dermatol
Perineal hidradenitis suppurativa: presentation of two unusual complications and a review
Ann Plast Surg
Axillary hyperhidrosis, apocrine bromhidrosis, hidradenitis suppurativa, and familial benign pemphigus: surgical approach
Objective scoring of hidradenitis suppurativa reflecting the role of tobacco smoking and obesity
Br J Dermatol
Surgical management of massive perianal hidradenitis suppurativa
Ann Plast Surg
Morbidity in patients with hidradenitis suppurativa
Br J Dermatol
Quality of life in hidradenitis suppurativa
Hidradenitis suppurativa of the perineum, scrotum, and gluteal area: presentation, complications, and treatment
J Natl Med Assoc
Axillary hidradenitis
Arch Surg
A case of dissecting cellulitis and a review of the literature
Dermatol Online J
Follicular occlusion triad: hidradenitis suppurativa, acne conglobata, and dissecting cellulitis of the scalp
Ann Plast Surg
Spondyloarthropathy associated with hidradenitis suppurative and acne conglobata
Ann Intern Med
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Hidradenitis suppurativa: Basic considerations for its approach: A narrative review
2021, Annals of Medicine and SurgeryCitation Excerpt :Studies of HS in children are associated with premature adrenochemia or precocious puberty, when androgens are dominant. As in acne, there are no increases in serum androgens in most HS19 patients and the effect is assumed to be caused by end-organ sensitivity [11–13]. Smoking: Smoking is a factor that is strongly associated with HS.
Gender differences in sexual health impairment in hidradenitis suppurativa: A systematic review
2021, International Journal of Women's DermatologyClindamycin versus clindamycin plus rifampicin in hidradenitis suppurativa treatment: Clinical and ultrasound observations
2019, Journal of the American Academy of DermatologyThe Treatment of Genitoperineal Hidradenitis Suppurativa: A Review of the Literature
2019, UrologyCitation Excerpt :Stage II progresses to recurrent abscess with evidence of tract formation and cicatrization. Stage III reflects almost diffuse involvement or multiple interconnected abscesses and sinus tracts across an entire region.3,4 Although it is based purely on visual manifestation of disease, the Hurley staging system may help in defining severity and thus aid in determining treatment modality.
Surgical management of hidradenitis suppurativa: procedural trends and risk factors
2018, Journal of Surgical ResearchHidradenitis suppurativa
2019, Piel