hidradenitis suppurativa(hs) acne inversa suppurativa(hs) acne inversa ... severe cases –...
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Hidradenitis Suppurativa(HS)Acne Inversa
Hope K. Haefner, MD
Conflicts of interest
Advisory Board, Merck Co., Inc.
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OBJECTIVES
1. Identify the clinical features of hidradenitis suppurativa (HS
2. To develop an effective therapeutic approach for HS, including surgery
Women 3.3:1
Prevalence 1-4%
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DefinitionChronic, inflammatory, recurrent, debilitating, follicular skin disease that usually presents after puberty with painful, deep-seated, inflamed lesions in the apocrine gland-bearing skin of the body, most commonly the axillary, inguinal and anogenital regions.
Second International Conference on Hidradenitis Suppurativa, March 5, 2009 San Francisco CA USA
Hidradenitis SuppurativaMisdiagnosed as “boils”
Mild cases - an inflammatory,folliculopustular disease
Severe cases – hypertrophic scarring, nodules, sinus formation
Profound impact on quality of life
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Pathogenesis
• Genetic predisposition; autosomal dominant
• Weak walled follicles, poor support
• Hormonal stimulation of ductal wall lining cells
• Blockage with subsequent follicular rupture
• Heat, sweat, irritation, squeezing may initiate
• Innate immune reaction to follicle contents
Multifactorial
Role of Androgens and Diet Ovarian, adrenal androgens Dietary androgens
Dairy is a triple threat
- raises the level of insulin (whey)- raises the level of IGF-1 (casein)- provides androgens and 5a-reduced
precursors of DHT in milkHigh glycemic load food- raises the level of insulin
Exogenous androgens – OCPs, implants, IUD, MPA injections
Controversial
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Diagnostic Criteria for HS
1. Typical lesions:Primary lesions - painful deep-seated nodules (blind boils)Secondary lesions - abscesses, draining sinuses,
bridged scars, “tombstone comedones”
2.Typical localization:Axillae and groin, genitals Under breasts, on buttocks and perineum
3. Chronicity and recurrences –Chronic recurrent lesions for more than six months
All three criteria needed for diagnosis
HS - Diagnosis
HS is often not recognized and diagnosis may be delayed for years
Result of missed / ignored Dx is devastating poor quality of life withpain, impaired function,wasted time and money,frustration and depression
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Factors that Worsen HS
Drugs – lithium, androgenic meds, progestins e.g. Mirena IUD,
Depot medroxyprogesterone acetate Smoking Stress Friction – any rubbing, squeezing, pinching Obesity
HS Clinical features
Chronic lesions
“tombstone comedones” – open twin or multi-headed comedones, left behind after sebaceous units
destroyed
chronic draining, malodorous sinuses
dense fibrous scars
pitted and pocked acne-like scars
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Hurley’s Criteria for HS Staging
Stage I: abscess formation, single or multiple,
without sinus tracts and cicatrization/scarring.
Stage II: recurrent abscesses with sinus tracts and
scarring, single or multiple, widely separated lesions.
Stage III: diffuse or almost diffuse involvement,
or multiple interconnected tracts and abscesses across the entire area.
70%
26%
4%
Stage I HS
Axilla
Rt inguinal crease
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Stage II HS Lesions
Chronic, recurrent lesions at same site, coalescing with fibrosis and sinus formation.
Lesions persist for months withpain and drainage
with foul odor
HS Stage II
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Hurley Stage II
Hurley Stage II
Stage III HS Lesions
Hypertrophic fibrous scarring with“bridged scars” forming rope-like bands with
active, painful, inflammatory nodules and sinus tracts forming thick scarred plaques over an area
Resulting in lymphedema and/or decreased mobility
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HS Stage III
HS Treatment Principles
Choose treatment to fit disease severity
A permanent cure is only with wide surgical excision(this concept needs to be reviewed)
Combine medical and surgical Rx
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Treatment HS
All Four Wheels on the Therapeutic Wagon
1. Hormonal Management Including Diet
2. Inflammation control
3. IPGM and sinus elimination
4. Comedone control
This is NOT ‘step therapy’
These measures are concurrent and parallel
and likely lifelong.
General HS Treatment for ALL Cases
Education – long time horizon, realistic goals
Zero dairy, low carb diet and support
Stop smoking
Reduce stimuli to rupture of pores : Reduce friction heat, sweating, obesity Loose clothing, boxer-type underwear Tampon use if appropriate / avoid pads
No picking, squeezing
Anti-inflammatories – antibiotics, corticosteroids, biologics
Anti-androgen Rx if possible
Metformin – for metabolic syndrome and obese
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Zinc and Vitamin C Supplement
• Used as anti-inflammatories and for wound healing
• Dose – Use zinc 50 mg / 2 mg copper amino acid complex
and vitamin C 500 mg bid
• Side effects – GI upset, nausea, diarrhea
Note: this avoids hypocupremia (blocks copper) that can cause
sideroblastic anemia, leukopenia, neutropenia
Inflammation Control in HSAntibiotics
As anti-inflammatory agents (almost all)
As antibiotics when there is infection
Anti-inflammatories
Classic corticosteroids – systemic, IM and IL
Biologics – TNF blockers and others
Other
Stop smoking
Stop friction, trauma to areas
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Biologic Rx for HS
Outcome – control not cure
• HS cases treated with biologics: infliximab, adalimumab, ustikinamab
• Most information on infliximab• For severe/extensive HS for control, eg. before surgery• Can be life-altering• Variable long-term results and significant adverse effects
Surgery - Clear IPGM and SinusesMini-unroofing
Punch biopsy 4 – 8 mm size,debride, seal with ferric chloride & petrolatum
Unroofing / Deroofing
Scissors, gauze, curette, CO2 laser, scalpel,ferric chloride, electrodesiccation
Surgical excision
• Classic with primary closure, flaps, grafts
• Extensive with wide excision, fenestrated split thickness grafts, VAC Dressings or secondary intent healing
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HS Surgery
• Stage III HS – This is a surgical conditionUse a knowledgeable surgeonStaged procedures work wellClosure: flaps, grafts, or by secondary intention
• “Recurrences” are mostly due to new lesions
• Patient satisfaction rates are very high
Mini-unroofingor
Punch debridement
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Un-roofing without preparation with biologic
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Hurley Stage III
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Split Thickness Skin Grafts
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Split Thickness Skin Graft for Hidradenitis Suppurativa (website)
(on website)
4 Months Post Op Grafts
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2 Years After Surgery
Courtesy Dr. Hope Haefner
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SummaryThorough exam and staging
Education, chronicity, expectations, support No smoking, hygiene/pressure relief, diet, metforminHormones - anti-androgens as indicatedAnti-inflammatories – topical and systemic antibiotics,
cortisone, depending on severityEarly unroofingFurther surgery as indicatedConsider biologics for severe HS
- for control before surgery
Resources
hs-foundation.org
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Disclosures
E.J. Hope K. Haefner, MDMerck & Co
- Advisory Board