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Tailor-made care

Experts discuss the benefits of personalized treatment plans for patients with hidradenitis suppurativa


Tailor-made care

Experts discuss the benefits of personalized treatment plans for patients with hidradenitis suppurativa


By Jan Bowers, contributing writer

Hidradenitis suppurativa (HS) was once considered a rare disease, thought to originate from inflammation of the apocrine glands. Some patients were made to feel that poor hygiene was aggravating their disease. Many patients and their dermatologists grew frustrated as they struggled to find effective treatments. All that is starting to change, thanks in part to a better grasp of the pathogenesis of HS, coupled with FDA approval of adalimumab for its treatment. “For dermatologists, hidradenitis is probably one of the worst diseases that we treat in terms of its impact on quality of life,” said Christopher Sayed, MD, associate professor of dermatology and director of medical student education at the University of North Carolina School of Medicine. “It’s relatively common, with estimates that it affects around 1% of the population. When adalimumab was approved as a treatment, that brought a lot of attention to it. But before that, it had been very much a neglected disease, with relatively little research into treatment. Those of us who treat a lot of HS patients and care about them feel that the new focus on the disorder is way overdue.” 

HS patients are predominantly female (by a ratio of three to one), of African descent, and of lower socioeconomic status. Dr. Sayed remarked that “for most patients, HS is going to start in the teens and 20s, remain active in the 20s and 30s, and then, for a lot of patients, taper off as time goes on. But it can persist decades beyond that.” HS is linked to increased risk of metabolic syndrome and obesity; about 75% of HS patients smoke. Dr. Sayed also pointed to associations with severe nodulocystic acne and polycystic ovarian syndrome.

Dr. Sayed co-chaired a group of physicians who recently published North America’s first comprehensive set of clinical guidelines for the treatment of HS (J Am Acad Dermatol. 2019;81(1):76-101). Acknowledging that the shortcomings of available evidence “make drawing comparisons between treatment options challenging,” the guideline authors evaluated the efficacy of surgical modalities, methods of pain management and wound care, light-based and laser treatments, topical and intralesional treatments, and systemic agents in the management of HS.

DW spoke with four dermatologists who have extensive experience in treating patients with HS. They emphasized that the treatment plan must be customized to the patient and offered their views on the latest advances in the understanding and management of HS.

Pathogenesis

HS is now understood not to be a disease of the apocrine glands, but a chronic inflammatory disorder that affects hair follicles, particular those in intertriginous areas such as the axilla, groin, genital, perineal, and inframammary regions. Increased levels of inflammatory cytokines such as TNF-alpha, IL-1, IL-17, and IL-23 in the skin of HS patients parallel those seen in psoriasis and rheumatoid arthritis, Dr. Sayed pointed out. “These are things that probably drive the disease. But what is the initial insult that kicks things off? That’s the major mystery. Not just that, but what are the most important cell types that are involved in this disease? We see a lot of neutrophils on histologic exam, but early on in the disease, before the follicles have ruptured, it may be something like a T-cell that is actually much more important.” More than half of HS patients have a family history of the disease, Dr. Sayed noted, “but the genetics are poorly understood. We really don’t know what it is that predisposes patients from a genetic standpoint.”

Investigation of the role of complement proteins in HS is also revealing potential new avenues for treatment, Dr. Sayed noted. When they act against bacteria, complement proteins may draw other inflammatory responses, sparking an inflammatory cascade that drives the inflammation further, he explained. “The thought is that perhaps in HS, because complement levels are much more elevated than they typically are, that maybe there’s some response to just even normal bacteria that’s happening in these patients’ skin that leads to very exaggerated complement-mediated response. If you can inhibit how complements act, you may be able to specifically block that part of the trigger.”

Assessment and acute care

For the clinician, the most useful tool for assessing the severity of HS and planning therapy is likely Hurley staging, according to the guidelines. Patients with Hurley stage I disease have recurrent nodules and abscesses with minimal scarring; stage II, one or a few sinuses and/or scarring within a body region; and stage III, multiple or extensive sinuses and/or scarring.

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What Hurley staging doesn’t measure are the factors that can drive patients into the ER, or into hiding: pain, abscess drainage, and odor. “As dermatologists, we need to be aware that pain has an enormous impact for HS patients. Controlling the drainage and the odor are also really important aspects of disease management,” said Joslyn S. Kirby, MD, associate professor of dermatology at Penn State College of Medicine and a co-author of the guidelines. “The impact of these things on patients’ quality of life and social interactions cannot be overstated. A lot of people with HS who I talk to feel very isolated by their disease. The pain can change the way they walk, sit, and reach for things. The stain and odor of the drainage can be so embarrassing that patients avoid social situations, fearing what may happen.” Dr. Kirby noted that incision and drainage of a painful abscess relieves the pressure and pain, “but that lesion will not resolve. It may get better, but it’s very likely to come back.”

For wounds that are open and draining, one dermatologist’s preferred treatment is to apply wash cloths soaked in diluted white vinegar to avoid burning. “This decreases the smell of the bacterial colonization, and then I apply dressings (e.g., calcium alginate, hydrofibers) that can contain the drainage at least for the day, so the patient is more comfortable,” noted Alex Ortega Loayza, MD, associate professor of dermatology at the Oregon Health & Science University who specializes in treating patients with HS and pyoderma gangrenosum. “People sometimes forget that it’s important to treat the ulcers or wounds at the same time we’re treating inflammation with medical therapy. The drainage is what bothers the patient, and if we treat the inflammation without treating the wound, we will have a cycle of persistent bacterial colonization or superinfection.”

Pharmacologic treatment

Topical antibiotics have not been shown to be effective against HS lesions, but many dermatologists turn to oral antibiotics as first-line therapy. “People use them in many ways,” said Dr. Sayed. “Probably minocycline and doxycycline are the most common because we use them all the time for things like acne and rosacea — not because there have been great studies that show them to be better than other antibiotics. The combination of clindamycin and rifampin [both used at a dose of 300 mg twice daily] are sort of the next step up. There have been a number of prospective and retrospective studies that have shown some benefit.” Dr. Ortega remarked that microbiome studies have identified different types of bacteria between the lesional and non-lesional skin of HS patients, “so that’s telling me that antibiotics should help correct the microbiome which has been altered in patients with HS. At the same time, it will also help treat infections which have been superimposed because of having chronic open areas. That’s why it’s one of the first treatments we use.”

Although limited evidence exists to support the use of hormonal therapies, the guidelines recommend that hormonal agents be considered “as monotherapy in females with mild-to-moderate HS or as adjunctive agents for more severe disease.” Hormonal treatment may be especially beneficial for patients who experience HS flares around menses, or those with polycystic ovarian syndrome. Dr. Ortega said that while his first step in medical therapy is to control inflammation, he also addresses the metabolic and hormonal components. “If patients are obese, they tend to have insulin resistance. For these patients I use metformin, which can decrease the metabolic component and inflammation. In addition, androgens are normally involved in follicular inflammation, and that’s why in some patients we use spironolactone.”

Immunomodulation with biologics is “rapidly becoming the cornerstone of therapy” for patients with moderate-to-severe HS, according to the guidelines. Adalimumab, a TNF inhibitor, is currently the only biologic approved to treat HS, but several others are under investigation, including infliximab (also a TNF inhibitor, sometimes used off label for HS), etanercept, golimumab, anakinra, and ustekinumab. “In my mind, the decision for starting someone on a biologic is they have proved recalcitrant to other things such as antibiotics or spironolactone, or I am looking at someone who has very severe and scarring disease,” said Dr. Kirby. “For patients in that [scarring] category, I very often will turn to a biologic as first-line therapy, because I’m concerned that an antibiotic or hormonal agent just wouldn’t be sufficient.” Another factor in favor of biologics, she added, is that “this is a chronic disease, and we need to find treatments that keep people under control without resorting to years and years of antibiotics.”

Adalimumab may also be appropriate for some patients with milder disease — a recommendation in the guideline based on expert opinion. For these patients, if “it’s very clear that it’s active disease and at risk of progression, and if they failed to respond to other lines of therapy, it wouldn’t be unreasonable to use it for some of those patients as well,” said Dr. Sayed. “Ideally, you don’t let patients get to the point that they have the scars and tunnels before you shut the disease down. Then you’re sort of stuck thinking more about procedures at that point.”

Although biologics are a promising treatment for HS, they’re not a silver bullet. Dr. Ortega pointed out that clinical trials of adalimumab indicate that “in about 50% of patients, at least 50% of the lesions in those patients improve. That means there are other cytokines involved, and that’s why studies of other agents are ongoing. My personal prediction is that in the next five to 10 years, we might be able to choose a medication based on personalized medicine, using what we learn from this research.”

Surgical and laser treatment

The nd:YAG laser can be used concurrently with medical therapy, or as monotherapy in patients with early disease who dislike using medications, said Dr. Sayed. “There have been some small studies that have shown disease improvement with laser hair removal,” he noted. “The problem is that in areas like under the breasts there are very small, lightly pigmented hairs that don’t respond to a laser. Once a patient’s disease has become more advanced and they have scarring and tunnels, the problem is no longer localized to the hair follicles, and the laser can’t remodel those deeper structures.” Some patients who have stage III disease in one area and milder disease in another can benefit from laser treatment in the less severe area, Dr. Sayed added.

The painful lesions and sinuses (tunnels) that characterize stage II and III disease generally require surgical treatment. Sometimes they resolve on their own with medical therapy, but in many cases scarring around a sinus or “an epithelial lining, much like you would see in skin within the hair follicle, makes it self-sustaining, even if the inflammation is turned off,” Dr. Sayed explained. The guidelines note that “when procedures are indicated, medical therapy may be initiated or continued without interruption.” Dr. Ortega said that before surgery for wide local excisions, he makes sure the patient is “medically controlled, meaning that I try to get the patient improving from, say, two to three lesions/flares per week to one to two per month. Then I’m confident sending the patient to surgery because I know the inflammation component is under control. The wide excision will be less invasive.”

Two surgical procedures available to dermatologists for HS, wide excision and deroofing, have both proven effective in controlling disease. One study cited in the guidelines showed a 24.4% recurrence rate for both procedures. “I really like deroofing for exploring the extent of the disease and not going beyond the tissue that’s being affected by HS,” said Dr. Kirby. “I choose excision for spots that have demonstrated recurrence after one of the more minor procedures such as deroofing or marsupialization. I’ll also do an excision if there’s a dense network of tunnels.” In both procedures, the wound is left open to heal. “Sometimes we will trim the edges in a deroofing to force it to heal over a longer period of time,” Dr. Kirby explained. “It sounds strange, but we don’t close the wound, since primary closure is associated with a higher risk of recurrence. It’s very important to make sure the patient really understands that the wound is going to look longer and wider, and remain open.”

Dr. Kirby emphasized that general dermatologists can perform these procedures. “If you can curette a basal cell, or excise a melanoma, the size and complexity of HS procedures is very similar to what you’re already doing in your practice. We can still work with our plastic surgery colleagues for broader, more complex procedures or those that require sedation.”

The chronic and debilitating nature of HS can often lead to depression, anxiety, and substance abuse, said Dr. Kirby. “I think as we get better and better as a medical community at recognizing this disease, validating it as a disease, and starting effective therapies earlier, we will make a huge impact on all of the comorbidities, including the psychosocial ones.” Dr. Sayed echoed that sentiment, noting that “a lot of physicians have trouble getting motivated enough to engage with these patients when they’re struggling a lot. They feel they run out of options very quickly. However, if you’re creative and stick with it, you can make a big difference for these patients and help guide them to appropriate treatment, even if you’re sometimes not the person who can appropriately administer it.”