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Hidradenitis suppurativa (HS) is a chronic immune-mediated inflammatory skin disease characterized by abscesses and inflammatory nodules, and occasionally tunnels and scars, in the axillae, groin, and inframammary areas.
Objective
HS can be challenging to diagnose because it mimics localized soft-tissue infection. The process of differentiating HS from soft-tissue infection is discussed. Patients with HS frequently visit emergency departments (EDs) for acute management of pain and drainage from HS lesions. This review updates emergency and urgent care physicians on how to educate and initiate treatment for patients with HS, and to coordinate care with dermatologists and other physicians early in their disease course.
Discussion
Recent updates on the epidemiology, diagnosis, and management of HS are reviewed.
Conclusions
Practice variations between how care for HS is provided in the ED setting and what HS treatment guidelines recommend are identified.
Patient present frequently to the emergency department (ED) with the typical signs and symptoms of a skin or soft-tissue abscess (e.g., localized warmth, redness, tenderness, and fluctuance). Approximately 3.28 million cases were diagnosed in 2005, comprising 2.9% of ED visits (
). The etiology for most of these patients is localized soft-tissue infection (coded as carbuncle, furuncle, or abscess). A potential clinical mimicker of soft-tissue infection is hidradenitis suppurativa (HS), an immune-mediated inflammatory disease that can have acute periodic flares superimposed on a chronic course. The typical scenario in which a patient with HS presents to an ED or urgent care is for the acute onset of new or worsened inflammatory nodules, abscesses, or swollen draining tunnels (Figure 1). Patients typically report severe pain, purulent drainage, and concern about possible infection. Approximately one-half of patients with HS also experience prodromal systemic symptoms of fatigue, malaise, fevers or chills, headache, or nausea prior to skin flare (
Figure 1Hidradenitis suppurativa (HS). Confluent abscesses with scarring on the left breast, lower outer quadrant. A bridging scar, which is specific for HS, is denoted with an asterisk.
). Because HS is less common than infection yet mimics it clinically, HS can be misdiagnosed as infection. This can contribute to a 7- to 10-year delay between symptom onset and diagnosis (
). Efforts to shorten the time to diagnosis and optimize both acute and chronic management would improve patient outcomes and reduce health care costs. In this narrative review, we provide an overview of the epidemiology, diagnostic criteria, and management of acute manifestations of HS and subsequent follow-up care outside the ED or urgent care. This review includes specific recommendations for diagnosing and treating HS based on up-to-date information. Through understanding the unique characteristics of HS and the importance of early recognition, follow-up care can be arranged to minimize future exacerbations.
Discussion
With an estimated 131,430 ED visits for HS in the United States in 2017, and an overall age- and gender-adjusted prevalence of 0.1%, HS is not a rare disease (
). Patients with HS frequently use the ED for care; a retrospective claims analysis found that adult patients with HS had a mean of 3.5 visits per person over 5 years (
). Recent increased disease awareness may stem from the approval of adalimumab as the first U.S. Food and Drug Administration–approved therapy for patients with moderate to severe HS (
). Incidence and prevalence are highest for women and African Americans. HS is a chronic condition with a median duration of 22 years after diagnosis (
). In addition, patients with HS have higher risk of several comorbidities, including metabolic syndrome, polycystic ovary syndrome, psychiatric disorders (e.g, depression, anxiety, and suicide), inflammatory bowel disease, and spondyloarthritis (
). Cigarette smoking and obesity are also associated, but there is no robust evidence that cigarette cessation or weight loss are consistently effective interventions to lessen HS disease activity (
HS is a diagnosis of exclusion that can be challenging to make in some circumstances. The principal diseases to be included in the differential diagnosis for HS include cellulitis, infectious abscess, epidermal inclusion cyst, or cystic acne. Although noninfectious, an HS abscess has many of the clinical hallmarks of an infection, such as erythema, warmth, swelling, and tenderness. This creates a challenge to differentiate it from infection without a history of multiple episodes. Valuable clues to aid in differentiating HS from infection are localization in skin areas prototypical for HS (e.g., axillae, inframammary folds, pannus, groin, and buttocks); presence of comedones in these areas; and a history of recurrence or signs of prior episodes, such as scarring at the site. Lymphatic streaking is not a feature of HS, so its presence should prompt wound culture to check for pathogenic bacteria (e.g., methicillin-resistant Staphylococcus aureus or group A Streptococcus). Patients reporting a history of two or more “boils” in the prototypical areas during the preceding 6 months have an HS probability of 0.85–0.89 (Box 1) (
). Rarely, patients with HS may have other associated follicular occlusion disorders, such as dissecting cellulitis of the scalp and acne conglobata (follicular occlusion triad) or pilonidal sinus (follicular occlusion tetrad). If these other follicular occlusion diseases are observed, this should help differentiate HS from infection. An epidermal inclusion cyst typically has been present for months to years prior to rupture and is usually solitary. There is typically a dilated follicle present in epidermal inclusion cysts (Figure 2), which is not observed in HS abscesses. Cysts may extrude an off-white material with the consistency of soft cheese, and HS abscesses emit purulent drainage. Cystic acne is differentiated from HS because it is more commonly localized to the face and torso. Routine skin culture and laboratory testing have no role in diagnosis or management of HS. Imaging with point-of-care ultrasound (POCUS) may visualize anechoic fluid-filled collections in the soft tissue if abscesses are present, and may show signs of chronic disease (e.g., interconnected sinus tracts), but there is no evidence that POCUS can reliably differentiate infectious abscesses from HS abscesses (
Two considerations in objectively assessing HS severity are the degree of current inflammatory activity and degree of cumulative damage, which reflect an integration of the consequences of past episodes of inflammatory activity. There is no standard method to determine mild, moderate, or severe disease. Disease severity can be based on patient-reported outcome measures, such as pain or quality of life scales, as well as clinician-reported outcomes. A convenient approximation for differentiating mild, moderate, and severe inflammatory activity is the Hurley staging (Figure 3A–C) (
). In Hurley stage I, inflammatory nodules or abscesses occur as individual lesions rather than coalesce. In Hurley stage II, tunnels and scarring are present, but do not coalesce completely over a body site. In Hurley stage III, an entire anatomic site is engulfed in a conglomeration of tunnels, other inflammatory lesions, and scars. The Hurley stage is typically used to describe a patient rather than each site, so all body sites are evaluated and the highest stage is applied for that patient. For example, if a patient has HS in their bilateral axillae and one side is stage II and the other is stage III, then the patient would be described as Hurley stage III.
Figure 3Hurley staging. (A) Stage I: papules and pustules with no scarring or tunnels in the right axilla. (B) Stage II: nodules with scars and tunnels interspersed with normal skin in the right axilla. (C) Stage III: nodules embedded in confluent scars and tunnels in the right axilla.
Treatment of flares can be medical or procedural. The most practical procedural intervention that can be employed in the ED or urgent care setting is incision and drainage (I&D) of an abscess. A repeated cross-sectional study of National Hospital Ambulatory Medical Care Survey data revealed that from 2006 to 2017, I&D was performed at 28.9% of ED visits for HS (
). Key points related to I&D for HS abscesses are provided in Box 2. Release of the fluid, often a mixture of pus and blood under pressure, reduces pain immediately. However, nonfluctuant lesions are not expected to improve after I&D. Contraindications to I&D include the presence of a pulsatile mass or proximity to vasculature or nerves (
). It is also crucial to set expectations, as I&D can reduce symptoms, but is not a cure for HS lesions because there is a significant recurrence rate (
). An alternative method is to use a punch biopsy tool instead of a scalpel to create a larger aperture (Figure 4A–D), which facilitates more complete and ongoing drainage of the contents (
). Emergency or urgent care physicians familiar with how to punch deroof HS lesions may employ this technique for recurrent abscesses, which requires more time than I&D, but has a lower risk of recurrence (
). The skin overlying a recurrent nodule or abscess is incised with a punch tool, then the purulent or gelatinous material within is removed, either with direct pressure or using a curette, and the site is allowed to heal by means of secondary intention (
). Continuing medical therapy, which may include antibiotics or biologic therapies, during the perioperative period may be associated with reduced recurrence risk (
Figure 4Using the punch biopsy tool to perform incision and drainage of an abscess in the right crural fold. (A) Punch biopsy tool presses into the abscess. (B) Purulent drainage is expressed. (C) Wound is gently patted dry with gauze. (D) A clean wound is dressed with petrolatum and gauze.
Regardless of the type of procedural intervention, HS abscesses should not be packed. For all procedural interventions, it is recommended that the site for the incision be anesthetized first (either locally or with field block, as appropriate). Local anesthetic efficacy is blunted by the inflamed tissue, but still gives patients some relief from the procedure-related pain (
). If feasible to administer, minimal sedation should be considered for patients with more severe pain or anxiety. Procedures also play a role in long-term maintenance therapy because persistent lesions are less likely to recur after more extensive excision or deroofing procedures (
). Because HS is a systemic disease, surgical excision of one area does not prevent or treat disease activity in other areas.
There are also several medical interventions that can be helpful to relieve an acute flare. For a single lesion or a few acute lesions, injection of triamcinolone intralesionally (10 mg/mL, 0.2–2 mL per lesion) into or immediately adjacent to painful lesions can reduce signs and symptoms of inflammation, often within 1–2 days (Figure 5) (
). Multiple studies have demonstrated that steroid injection can treat flaring HS lesions rapidly, which supports our current conception of this disease as having an inflammatory rather than infectious etiology (
). If the triamcinolone is injected directly into an abscess that communicates with the skin surface, the injected material may leak. Repositioning the needle deeper or adjacent to the abscess may permit further injection without leakage. If there is no leakage, adding the volume of triamcinolone will swell the abscess and may aggravate abscess-associated pain, which is the signal to cease injection. Oral antibiotics can be helpful if many lesions are inflamed or adjunctive therapy to intralesional injection is warranted. The skin microbiome is dysbiotic in HS, but this may be a consequence of the inflammation, so cultures are not performed routinely (
). Antibiotics can also be used for their anti-inflammatory properties (e.g., tetracyclines can suppress neutrophil migration and chemotaxis) or for their ability to modulate dysbiosis (
). Oral antibiotics are prescribed at discharge from 66.6% of ED visits, with clindamycin (38.6%), trimethoprim-sulfamethoxazole (37.2%), and cephalosporins (28.2%) prescribed most frequently (
). These results suggest that there may be a practice variation between what antibiotics are prescribed at ED visits and what is recommended in HS guidelines (
North American clinical management guidelines for hidradenitis suppurativa: a publication from the United States and Canadian Hidradenitis Suppurativa Foundations: Part II: topical, intralesional, and systemic medical management.
). An antibiotic regimen that is convenient, inexpensive, and well-tolerated, with limited risk for drug–drug interactions and with evidence of efficacy in HS is doxycycline 100 mg twice daily (b.i.d.) (
). This can be used for both the flare and as the beginning of a maintenance therapy. A 12-week regimen of doxycycline 100 mg b.i.d. is a common therapeutic trial for HS. It may be most practical to initiate the prescription in the ED and discharge the patient from the ED with a 90-day prescription to a dermatologist or their primary physician to continue management until the patient secures a dermatology consultation. Doxycycline is contraindicated in pregnant patients and in patients 8 years and younger, except for treatment of severe or life-threatening conditions (
). Table 1 suggests some alternative oral antibiotic regimens if a patient is intolerant or has had an inadequate response to doxycycline. HS treatment with medications other than oral antibiotics has been reviewed recently, but these medications are not typically prescribed in the ED or urgent care setting (
North American clinical management guidelines for hidradenitis suppurativa: a publication from the United States and Canadian Hidradenitis Suppurativa Foundations: Part II: topical, intralesional, and systemic medical management.
Figure 5Intralesional injection in the right axilla. Needle of tuberculin syringe containing triamcinolone suspension inserted at the intradermal level into an inflamed nodule.
North American clinical management guidelines for hidradenitis suppurativa: a publication from the United States and Canadian Hidradenitis Suppurativa Foundations: Part II: topical, intralesional, and systemic medical management.
12 wk, or up to 12 mo if needed to bridge to appointment
Mild to moderate uncontrolled HS
Allergy to drug class, photosensitivity, gastrointestinal upset, Candida vulvovaginitis
Clindamycin + rifampicin
p.o.
300 mg b.i.d. of each
12 wk, or up to 12 mo if needed to bridge to appointment
Second-line treatment for mild to moderate disease or as a first-line or adjunct treatment in severe disease Clindamycin monotherapy is appropriate if rifampicin is contraindicated or causes an adverse event (
Pain is a cardinal symptom of HS, often disproportionately severe relative to the apparent degree of inflammation. Pain management includes disease-directed anti-inflammatory therapy and, adjunctively, analgesic therapy. HS pain can have nociceptive and neuropathic components. A proposed treatment algorithm for pain management in HS suggests that, after disease-directed management, acetaminophen and topical nonsteroidal anti-inflammatory drugs (NSAIDs) are first-line therapies for acute pain (
). If neuropathic pain is suspected because of symptoms of burning or tingling, topical lidocaine 5% cream or 4% patch may help. More severe or refractory pain is treated with systemic NSAIDs, assuming these are tolerable and there is no contraindication to their use (in which case acetaminophen or tramadol are more appropriate choices) (
). Although not specifically studied for control of pain in HS, a randomized controlled trial for management of acute upper extremity pain in the ED demonstrated that a single dose of ibuprofen 400 mg + acetaminophen 1 g was as effective at pain reduction as opioid + acetaminophen combinations (
). In the absence of contraindications, it is reasonable to recommend for post-ED visit analgesia that patients not exceed the recommended daily doses of ibuprofen (200–400 mg per os [p.o.] every 6 h [q6h], not to exceed 1200 mg/d) and acetaminophen (1000 mg p.o. q6h, not to exceed 3000 mg/d) (
). Ibuprofen and acetaminophen can be taken either simultaneously or alternating every 3 h. Patients with HS have higher rates of substance use disorders, so the HS pain management treatment algorithm suggests that opiate therapy should be used carefully and signs of addiction or abuse monitored (
). When necessary, in the acute setting, tramadol or another short-acting opioid (e.g., hydrocodone or oral morphine) may be selected for patients with pain that is severe or refractory to systemic NSAIDs, or when NSAIDs may be contraindicated (
). For any patient with pain severe enough to justify prescribing opioids, it is crucial to ensure follow-up to a specialist in dermatology or surgery who is familiar with the recurrent nature of HS and comprehensive treatment regimens because control of disease activity will address the pain at its source. The clinicians managing HS over the long term will often use a multidisciplinary approach, incorporating input from physical therapy, wound care, and behavioral health, with a referral to a pain management specialist.
In addition to addressing the acute needs of the patient with HS, the ED or urgent care visit is an opportunity to establish the diagnosis for undiagnosed patients and to set the patient on the path to long-term disease management with appropriate referrals. Bridge medications to reduce inflammation and alleviate pain sufficient to last until the next appointment should be prescribed. Unfortunately, little can be advised regarding steps patients can take to reduce their risk of disease flare because we lack an understanding of what induces disease flare. A surgery consult or referral may be needed for large (e.g., > 5 cm in diameter) or complicated abscesses (e.g., abscesses recurrent in scars), which may need to go to the operating suite to achieve proper analgesia. Referral to dermatology, when feasible, or other appropriate medical and surgical specialties, is appropriate for patients who experience flares regularly (i.e., more than once per month). Regular follow-up with a primary care provider is important to ensure that patients are monitored and, if necessary, managed for HS-associated comorbidities (e.g., metabolic syndrome, anxiety and depression, and inflammatory bowel disease) (
). Emphasizing to patients with HS that dermatologists can provide long-term medications proven to reduce the risk and severity of flares can help motivate patients to follow-up in the outpatient setting. Dermatologists are likely best suited to review the benefits and risks of different therapies, monitor therapeutic response, and arrange subsequent referrals, as needed. Dermatologists have the experience to prescribe and monitor for the adverse effects associated with tumor necrosis factor–α inhibitors, such as adalimumab or infliximab. Ultimately, holistic management of a patient with HS may entail referral to mental health specialists (to screen for and manage comorbid depression or anxiety), primary care (for screening and management of comorbidities, such as hypertension, diabetes mellitus, or hyperlipidemia), and pain management (if pain is recalcitrant). The Hidradenitis Suppurativa Foundation (www.hs-foundation.org) has a list of recognized HS specialty clinics organized by state. The psychological benefit of joining a well-vetted online HS support group is substantial, as many patients with HS feel ostracized because of their disease and benefit from sharing their experiences with fellow patients (see Box 3).
Box 2Tips for successful incision and drainage (I&D) of diagnose hidradenitis suppurative abscesses.
•
Hidradenitis suppurative abscesses should be anesthetized with local anesthetic prior to I&D
•
I&D should only be performed on acute abscesses, not on chronic tunnels
•
I&D provides immediate pain relief, but recurrence rates are high
•
Abscesses should not be packed
•
Creating a larger skin opening using a punch biopsy instrument allows continued drainage after the procedure
The diagnosis of HS may be delayed because of its clinical similarity to the more common localized soft-tissue infection. Providers in the ED or urgent care play a critical front-line role in expediting diagnosis, managing flares, and assuring that patients with HS receive the appropriate follow-up to manage their disease long-term. These interventions increase the likelihood that the painful, recurrent, scarring trajectory of the disease can be altered beneficially.
Article Summary
1. Why is this topic important?
Hidradenitis suppurative (HS) is a painful, recurrent, scarring disease that impairs quality of life considerably. As many patients with HS are diagnosed or seek care in the emergency department (ED) or urgent care setting, a review of recent information about the epidemiology, diagnosis, and management of HS should improve HS patient outcomes.
2. What does this review attempt to show?
Differentiating HS from clinical mimickers is generally straightforward. Acute interventions in the ED can substantially alleviate HS morbidity in the short-term.
3. What are the key findings?
Incision and drainage is a common surgical intervention for acute lesions in the ED, but outcomes using a punch biopsy tool may be better. The recommended first-line oral antibiotic is doxycycline. Opiates should be used sparingly for pain management.
4. How is patient care affected?
Providers in the ED or urgent care can expedite HS diagnosis, effectively manage disease flares, and assure that HS patients receive the appropriate follow-up to manage their disease long term. The interventions suggested in this review increase the likelihood that the painful, recurrent, scarring trajectory of the disease can be beneficially altered.
Acknowledgments
Noah Goldfarb, MD provided critical review of the manuscript. Steven Daveluy, MD provided the photographs in Figures 3B and 3C. Chris Sayed, MD provided the photographs in Figure 4.
References
Taira BR
Singer AJ
Thode HC
Lee CC.
National epidemiology of cutaneous abscesses: 1996 to 2005.
North American clinical management guidelines for hidradenitis suppurativa: a publication from the United States and Canadian Hidradenitis Suppurativa Foundations: Part II: topical, intralesional, and systemic medical management.
Martin M. Okun has been a consultant for AbbVie, Azora Therapeutics, Bluefin Biomedicine, Boehringer Ingelheim, Chemocentryx, Gilead Sciences, Glaxo Smith Kline, Incyte, Innovaderm, InflaRx, Genentech, Pfizer, Phoenicis, Regeneron, Seattle Genetics, and Vyne Therapeutics. Joslyn S. Kirby has been a speaker for AbbVie and a consultant for AbbVie, Bayer, ChemoCentryx, InflaRx, Incyte, Janssen, Novartis, and UCB. The remaining authors disclose no conflicts.
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