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Chronic relapsing process with purulent material and scarring in the apocrine follicles.
Immunologic abnormalities, genetic predisposition, obesity, and smoking are important risk factors.
Pathogenesis of the disease has not been fully elucidated.
Histopathology suggests hyperkeratosis of the follicular epithelium as the primary event.
Likely results from synergistic effect of multiple factors.
Chronic inflammation results in scarring and complications including squamous cell carcinoma.
Reduces self esteem and quality of life.
Characterized by tender, deep nodules, abscesses, sinus tracts, and extensive scarring.
Has a tendency to become chronic with subcutaneous extension and sinus and fistula formation.
Chronicity and recurrences are hallmarks, yet there is substantial variability in its clinical course.
Associated with abscesses within the axilla and perianal a
More common in women with a ratio of 3:1.
Affects fewer than 200,000 people in US.
Estimated delay from diagnosis to diagnose is ranges from 7-12 years.
Prevalence estimated to be between 0.1% and 4.1%.
Familial and sporadic cases have been reported.
35-40% of patients report a family history.
70-90% of patients report smoking and more severe disease is noted in smokers.
Severity and course are correlated with body mass index
Mutations in the gamma-secretases-Notch signaling pathway, involved in epithelial proliferation and differentiation, has been identified in both familial and sporadic cases and plays an etiologic role in only a minority of cases.
Elevated levels of pro inflammatory cytokines-tumor necrosis factor alpha, interleukin-beta, IL-17, IL-10 in skin lesions.
May be associated with ab2242ant immune response to commensalism microbes with a production of anti microbial peptides and cytokines and recruitment of an inflammatory infiltrate.
Inflammation results in hyperkeratosis of the follicular infundibulum and subsequent follicular plugging, rupture and activation of the keratin fibers in the dermis.
Follicular occlusion is a nidus for bacterial colonization, which , may trigger an immunological response.
Disease most often appears after puberty commonly, in the second and third decades of life, and is rare in the elderly.
There is a strong female preponderance.
Usually occurs in the third decade of life.
Prevalence in industrialized counties to be 0.3-4%.
Among young adults being screened for sexually transmitted diseases point prevalences of up to 4% (Jemec GB et al).
More common in whites, blacks and uncommon in Asians.
Usually starts in the postpubertal age group.
The untreated process may persist into the seventh decade.
Manifests as painful, inflamed lesions, including nodules, sinus tracts, and abscesses.
Diagnosis is a clinical one with recognition of typical findings and course of disease.
Sites of involvement include axillary, inguinal, perianal, anal, perineal, mammary and inframammary, buttocks, pubic region, chest, scalp, retroauricular area and eyelid.
Severity of disease is graded by the Hurley staging system.
Three clinical subtypes exist:1- classic axillary-mammary subtype representing 48% of cases characterized by breast and axillary involvement and hypertrophic scarring 2- atypical subtypes a follicular subtype representing 26% cases predominately in male smokers with a family history and characterized by follicular lesions, including epidermal cyst, pilonidal cyst, comedomes, and severe acne and 3-a gluteal subtype representing 26% of cases typically smokers with a lower body mass index, follicular papules, folliculitis, and gluteal involvement.
Associated with intermittent flares with increased pain and suppuration.
Poorly controlled disease can lead to fibrosis and scarring with axillary disease leading to limb contractures and impaired mobility.
Complications of chronic suppurations include anemia, hypoalbuminemia, secondary amyloidosis, peripheral edema, arthropathy, fistula from nation in squamous cell carcinoma in the areas of chronic inflammation.
Diagnosis can be established by the chronicity and post pubertal onset, and physical exam of multiple inflamed lesions, symmetrical involvement, intertriginous predominance, tombstone
A diagnostic algorithm with 4 characteristics for diagnosis: more than a single inflamed lesion, chronic course with new and recurrent lesions, bilateral lesions, and the lesions primarily located in the milk line.
Differential diagnoses include: nodular acne, developmental fistula, epidermoid, dermoid, pilonidal, or bartholin cysts, infectious abscesses, carbuncles, furuncles, actinomycosis, cat scratch disease , granuloma inguinale, lymphogranuloma venereum, syphilis, and tuberculosis.
Skin manifestations of inflammatory bowel disease should be considered in the setting of perianal lesions and associated gastrointestinal symptoms.
Bacterial, fungal and mycobacterial culture can help in the differential diagnosis as these patients cultures are typically sterile or have multiple species as opposed to a single infectious agent.
Risk of squamous cell carcinoma 4.6 fold increase compared to non-affected individuals.
HS patients are typically afebrile
Psychosocial problems include impaired quality-of-life, decreased work productivity, depression, and social isolation.
Often occurs pre-menstrually in women.
Flares generally subside within 7 to 10 days, if untreated.
Perianal involvement more common in men.
Women more likely to have genitofemoral lesions.
The prevalence declines after age 50 years.
Sub-mammary, axillary and inguinal involvement more common in women.
Possible areas of involvement include the axilla, inguinal region, sub-mammary region, perineum, buttocks and scrotum.
Presumed to be related to folliculitis and local trauma.
Commonly associated with streptococci, staphylococci, E. coli, anaerobic bacteria and Proteus species.
Associated with improvement and worsening after pregnancy.
Some individuals with HLA-A1 and HLA-B6 have a predisposition to the process.
One third of patients report a family history, and affected families with an autosomal dominant mode of inheritance.
Occasionally the process is accompanied by severe acne and perifolliculitis capitis, linked to chromosome 1p21.1-1q25.3 and to mutations of the gamma-secretase complex (Wang B et al).
Some patients have a defect in T cell lymphocytes.
Associated with cystic acne, and pilonidal cysts.
The course is usually prolonged and manifests by intermittent episodes of activity and remission.
Lesions may rupture and develop dermal and subcutaneous sinus tracts.
Normal skin may be compromised by persistent draining sinuses and severe dermal scarring results.
May be triggered by smoking.
Smoking associated with severe disease.
Obesity is a risk factor.
Obesity compounds the severity of the disease through mechanical effects including sweat retention, mace ration, and enhanced mechanical friction, and possibly due to coexisting hormonal alterations.
In patients with HS who have undergone bariatric surgery 15% or greater reduction in weight is associated with a 35% decrease in symptoms and significant reduction in the number of involved sites.
Isolated reports of sustained remission have occurred with extreme weight loss or bariatric surgery.
May be triggered by use of lithium.
Androgens may play a role in pathogenesis suggested by premenstrual onset and female predominance, along with improvements during pregnancy or with anti-hormonal therapy.
Initially pathophysiology related to keratin comedones in the apocrine gland follicles, along with inflammatory cells and distended ducts filled with white cells and secretions.
Later bacteria are observed within the apocrine glands indicating the presence of infection and abscess formation.
Suppuration eventually extends to adjacent and subcutaneous tissues.
Nodules develop into pustules and rupture purulent material.
Onset is usually after puberty, in second and third decades when apocrine sweat glands start to develop.
Perineal disease tends to be severe.
Early lesions are solitary, pruritic and painful nodules and may last for weeks to months.
The nodules my undergo subcutaneous extension with indurated plaques.
Healing is associated with dense fibrosis and recurrence occur around the original site and leads to chronic sinus formation.
Associated with intermittent release of discharge of purulent and serosanguinous type.
Ulceration and abscess formation can occur and rupture into surrounding tissues.
Secondary cellulitis may intermittently occur.
Associated with poor general health.
Epidemiologic studies suggest a 50% increase in the risk of cancer of any kind, as compared with the general population (Lapins J et al).
Associated with specific cancers, including squamous cell carcinoma, buckle cancers and hepatocellular cancers.
Incidence increased among patients with Crohn’s disease, affecting 17% of such patients.
Relationship between Crohn’s disease and HS suggested by similarities and sinus tract formation, granulomatous inflammation, scarring, and onset after puberty.
Arthritis that is rheumatoid factor negative and HLA-B27 negative is more frequent among HS patients than in the general population, and usually involves peripheral joints.
A multifocal disease with atrophy of the sebaceous glands and subsequently early lymphocytic inflammation and hyper keratosis of the pilosebaceous unit, followed by hair follicle destruction and granuloma formation.
Subsequent healing processes produce scarring and sinus tract formation.
Suggested that IL-12-IL 23 pathway and TNF-alpha may be involved in the pathogenesis.
Diagnosis is a clinical one with characteristic inflamed and not inflamed nodules, sinus tracts with or without drainage, and abscesses located in the axilla, inguinal, and anogenital regions.
Lesions may involve the anus, buttocks, and female breasts as well.
Nodules are located in the deep dermis and appear rounded rather than pointed.
May be associated with secondary lesions such as open sinus tracts, plaque like in duration, pyogenic granulomas and multi headed comedones.
Greatest severity of the disease associated with male sex, disease duration, body mass index, smoking history, presence of axillary, perianal, or mammary lesions, personal history of acne , spontaneous/non familial disease, and involvement by atypical locations.
Skin biopsies and bacterial cultures are required only in atypical or refractory cases.
Bacterial cultures are usually negative, but flares may be associated with bacteria, including Staphylococcus aureus.
Diagnostic delay is common with the median delay of 12 years before diagnosis is made.
Lesions commonly misdiagnosed as common boils.
Staged on the basis of the Hurley staging system.
Hurley stages: stage I disease-localized and includes the formation of single or multiple abscesses, without sinus tracts and scarring.
Stage II-characterized by recurrent abscesses, with sinus tract formation and scarring, occurring as single or multiple lesions.
Stage III-diffusely nearly diffuse involvement of the affected region, with multiple interconnected tracts and abscesses.
Majority of patients present with stage disease.
Only 1% of patients progress to stage III.
Stage I disease is usually managed with topical therapy, whereas systemic therapy is recommended for more widespread or severe disease.
For early-stage disease topical clindamycin reduces the number of abscesses, nodules, and pustules during a three-month evaluation.
In women, antiandrogen therapy can sometimes be effective.
Antibiotics are mainstay of treatment, because of antibacterial and immunomodulatory properties.
Topical 1% clindamycin can be adequate treatment for mild disease, and adjunct therapy or a tool to decrease inflammation before radical surgery in severe cases.
Systemic antibiotics are effective for a large proportion of patients with mild to moderate but not severe disease.
Monotherapy with doxycycline, amoxicillin with clavulanic acid, clindamycin and other tetracyclines and ciprofloxacin are commonly used in the clinical setting, only combination therapy has been fully evaluated.
Rifampicin and clindamycin is the most widely studied regimen with a response rates ranging from 71 to 93% reported for 10 week courses of treatment, and a total of 11 to 47% of patients have complete remissions.
Isoretinoin is not effective therapy.
In severe cases immunosuppressive agents including cyclophosphamide, TNF-alpha inhibitors have been efficacious.
Quitting smoking and treatment for obesity are suggested management approaches, without controlled data.
Rubbing of the affected areas should be avoided.
Aggressive early diagnosis is important to control the disease because destruction of cutaneous tissue that accompanies this disease is difficult to treat and associated with debilitating medical and psychosocial sequelae.
In advanced cases with scarring, surgical options offer the best chance for cure.
Intralesional corticosteroids may be effective for individual lesions.
When topical treatment is insufficient oral tetracycline at 500 mg b.i.d. is commonly used, but does not have superiority over topical therapy.
Combination therapy with rifampin and clindamycin is associated with a reduction of disease severity by 50% (Gener G et al).
Surgery is reserved for unresponsive lesions.
Scoring is not responsive to medical therapy and surgery is a relative indication for such lesions.
The use of incision and drainage procedures is discouraged since recurrence is the usual pattern of response, and nonfluctuating nodules to not readily drain.
Surgical procedures include exteriorization of sinus tracts and localized excision.
Patients who undergo more extensive excision involving all hair-bearing skin in the infected area do better than those who have excision of inflamed lesions only (Mandal A, Watson, J).
Laser therapy has recently been utilized for treatment.
External beam radiation may be usrd, but rarely.
Bacterial superinfection in hidradenitis suppurativa does not initiate the disease, but it is one of the major contributors to an inflammatory vicious circle.
Bacterial infection plays a role in HS and antibiotics are part of maintenance therapies.
Hidradenitis suppurativa (HS), the majority have more than one bacterial isolate, with the most common isolates being Staphyloccus epidermidis, Proteus mirabilis, and Staphyloccus aureus.
Amoxicillin with clavulanic acid or fluoroquinolones are first-line antibiotic therapies followed by long-term maintenance with tetracyclines or macrolides due to their anti-inflammatory properties.
The majority of the patients have a polymicrobial flora with up to 5 species, predominantly staphylococci and bacteria of intestinal flora.