Hidradenitis Suppurativa in Pediatric Patients

Hidradenitis suppurativa is a chronic inflammatory skin disorder that primarily affects areas that contain apocrine glands, including the armpits, groin, and buttocks. It is reported in up to 2% of Western populations, with increasing incidence in children and adults.

Almost a third of hidradenitis suppurativa cases occur in pediatric patients and almost half of patients confirm initial symptoms in childhood.

To date, there are few clinical studies and guidelines for pediatric hidradenitis suppurativa. Here we review the epidemiology, clinical presentation, comorbidities and management of pediatric hidradenitis suppurativa. The barriers that contribute to delays in diagnosis and the important physical and emotional impact of the disease on children and adolescents are discussed.

Hidradenitis suppurativa (HS) is a chronic, inflammatory, and debilitating disease that primarily affects the skin with apocrine glands.

It presents with painful nodules and abscesses with a predilection for skin folds, which eventually form scars. It was first described in 1833 as abscesses in the axillary, mammary and perianal regions, and was treated mainly surgically.1

In 1854, it was erroneously related to sweat glands, which gave rise to its name (from the Greek Hydros for sweat and aden for glands).2,3 It was not until the 20th century that it was proposed that HS would be a primary inflammatory process of the apocrine glands.4,5 The complete pathophysiology of HS is still being elucidated.

Early identification of HS can be challenging as it depends on patients presenting with typical skin lesions, with a predilection for intertriginous sites and recurrent disease flares.6–8

Waiting for the typical waxing and waning course in pediatric patients can delay diagnosis, and the disease is commonly missed or confused with infection.9 Many patients with pediatric HS (PEHS) present to emergency departments with flares. of diseases that are controlled with antibiotics or incision and drainage, and the diagnosis is not suspected.

Studies have shown that the gap between the age of disease onset and the age at diagnosis of HSped is almost 2 years.10 Unfortunately, underrecognition leads to undertreatment, disease progression, and complications such as scarring. and contractures.10,11

The first 6 years of the disease are usually the most severe,6 so there is an early window of opportunity for aggressive treatment to avoid permanent damage. Thus, it is important to increase awareness of HS in primary, urgent and emergency care settings.

The exact prevalence of HS worldwide has been difficult to estimate due to misdiagnosis and diagnostic delay, although recent studies show rates between 0.1% and 2%.7,12

In the United States, the estimated overall point prevalence is 0.098%, with the highest prevalence being 0.17% in those between 30 and 39 years of age.13 The prevalence of HSped is estimated at 0.03%, but the point prevalence varies with age (0.11% between 15 and 17 years, 0.03% between 10 and 14 years, and 0.002% in children under 9 years).14 By far, the highest level of prevalence is observed in female adolescents. 15 to 17 years old who are African American or biracial (0.53% and 0.25%, respectively).13,14

HS was previously reported to occur most commonly between the ages of 20 and 24.15,16 More recent studies show a bimodal distribution, with a first peak in the late teens and a second peak in the mid-40s.17 Women experience an earlier onset of HS compared to men.17 Many adults report onset in childhood, with up to 50% of patients experiencing symptoms between 10 and 21 years of age.18

It is estimated that 7% of patients with HS develop symptoms before age 13 years and 2% before age 11 years.19 Patients with early-onset HS are twice as likely to report a positive family history of the disease and have more likely to be male.20,21

Studies have not associated early-onset HS with more severe disease. HS is a disorder with racial and ethnic disparities, with higher incidence, prevalence, and severity in blacks and Latinos.22,23 However, its prevalence is likely underestimated due to delays in diagnosis and limited access to care. specialized,21 possibly explaining why a more severe disease at presentation is found in the black and Latino population.

The pathophysiology of HS is complex. Smoking, obesity, friction, and different microorganisms are just some of the inciting events that are believed to exacerbate the process of follicular occlusion and dilation, subsequent follicular rupture with inflammatory response and cytokine activation, and profibrotic imbalance leading to sinus tracts. and scars in chronic HS.24

Early HS lesions are associated with T helper (Th) 1 and Th 17 type inflammation. 24 As the disease progresses, increases in the levels of interleukin (IL)-1, tumor necrosis factor (TNF), IL -17, calcium-binding protein A9 S100, caspase-1 and IL-10 in tissue, which are complemented by the influx of neutrophils, monocytes and mast cells.15

Dermal tunnels in chronic HS may be sources of inflammation, leading to increased expression of pro-inflammatory cytokines and chemokines such as CXC Motif chemokine ligand1 (CXCL1) and CXCL8 that drive local and systemic neutrophilic infiltration.25 Furthermore , imbalances in matrix metalloproteinases and tissue inhibitors of metalloproteinases increase profibrotic factors, such as tissue growth factors β 1, 2, and 3.24 This contributes to scarring and tunneling, hallmarks of HS.

Although HS is not a primary infection, the microbiome plays a role and superinfection is controversial.

The microbiome in the lesional and non-lesional skin of HS patients differs significantly from that of healthy controls.26 Bacterial spread within the intertriginous skin may stimulate immune activation.27 Distinct microbiological profiles have been identified in HS lesions, depending on the type and severity of the injury.28

An intriguing new concept proposes that HS could be considered “self-infectious” due to a strictly cutaneous immune dysregulation led by the host microbiome.29 Further characterization of the microbiome in patients with HSped is needed, as there may be differences with the Adults.

Sex hormones , particularly androgens, have been linked to the pathogenesis of HS.30 Support for hormonal involvement in the pathophysiology of the disease includes the observations that women are predominantly affected and may experience premenstrual flares. HS also tends to improve during pregnancy, postpartum flare, and is often exacerbated by oral contraceptives with a low estrogen:progesterone ratio.31 Additionally, HS is rare before puberty or postmenopause, and may be the presenting feature of premature adrenarche.32

Children with HS may be more likely to have hormonal imbalances compared to adults.33 However, no specific hormonal abnormalities or hyperandrogenism have been demonstrated in patients with HS despite extensive study, suggesting that hypersensitivity to Androgens may be more relevant locally at the level of the pilosebaceous unit.34

A family history of HS has been reported in up to 41% of patients.10,35 Syndromic HS has been associated with autosomal dominant mutations in the γ-secretase complex genes ( NCSTN, PSEN1, PSENEN, MEFV, POFUT1, PSTPIPP1 , and FGFR2 ), although these mutations are rare in the general HS population.29,36 These variants were initially linked to Notch signaling, which plays an important role in follicular differentiation, keratinization, occlusion, and cystic formation, but has not been identified a differential expression of Notch 1 to 4 in skin affected by HS.37 Genetic factors related to sporadic cases of HS are less understood and are the focus of active research.

HS is predominantly a clinical diagnosis.

In general, its presentation in pediatric patients is similar to that in adult patients. Lesions include inflammatory nodules, abscesses, ulcers, comedones, sinus tracts, and variable types of scars. Inflammatory nodules may be encapsulated or may rupture with foul-smelling discharge, which may be confused with an infection.

Patients may have comedones , with two-headed comedones being pathognomonic of HS. Patients may also experience superficial, painful ulcers similar to cutaneous Crohn’s Disease. In moderate to severe disease, painful fluctuating abscesses or tunneled sinus tracts predominate. Sinus tracts consist of multiple interconnected tunnels and are almost always associated with scars. Scarring includes atrophic or fish-mouth scars, hypertrophic, rope-shaped scars, and even contractures.

A multicenter review of 481 patients with HSped reported that cysts or abscesses (48%) and pain or tenderness (25%) were the most common signs at disease onset. Lesions on initial dermatological evaluation included cysts or abscesses (49%), pustules or papules (49%), nodules (20%), double-headed comedones (23%), sinus tracts (10%), and ulcers (6%).

The most commonly affected anatomical sites in HSped are axillae (75% to 85%), groin or inguinal folds (47% to 58%), and buttocks (6% to 27%), and most patients have 1 to 2 body sites involved.10,21,38 Axillary involvement is more likely to occur in men, while women have higher rates of inguinal involvement.39,40

Nearly half (48%) of patients with HSped already have scarring at the time of initial presentation to the dermatologist.10 Although this may be confounded by the observation that patients with aggressive disease are more likely to seek medical attention, physicians should be prepared or even expect to treat aggressive diseases in pediatric patients.10,16,41,42

Due to their age, pediatric patients are vulnerable to undertreatment, which can lead to scarring and significant psychosocial morbidity.

Delays in diagnosing HSped are common, ranging from 0.7 to 2 years.10,16,41,42 A comparison of claims data showed that patients with HSped were more likely to receive diagnoses of comedones and folliculitis than adults.16 It is unclear whether this reflects a difference in the early presentation of HSped or a true misdiagnosis.

Referrals to dermatology for HS are most commonly made by pediatricians (45%). Although emergency medicine accounts for only 5% of referrals for HS, up to 22% of pediatric patients report having visited the emergency department and 8% having been hospitalized.10 Targeted educational interventions can improve delays in diagnosis.

The Hurley stage scoring system is commonly used to assess the severity of HS (scoring systems used in adult HS studies are not validated for HSped). Hurley stage I encompasses single or multiple abscesses without sinus tracts or scars. Hurley stage II broadly describes one or several separate recurrent abscesses with sinus tract formation and scarring. Hurley stage III describes diffuse involvement with interconnected tracts and abscesses throughout the area.43 Between 35% and 53% of patients with HSped present with Hurley stage II or III disease.10,42

Hurley staging lacks quantitative data and dynamic evaluation over time. Other assessment tools exist, including the Sartorius Scoring System, the Hidradenitis Suppurativa Clinical Response, the Global Medical Assessment of Hidradenitis Suppurativa score, and the International Hidradenitis Suppurativa Severity Scoring System.

A dynamic scoring system is important in pediatric patients as they may experience rapid disease progression regardless of initial Hurley staging.41 Current clinical trials use Hurley stages and nodule counts for inclusion criteria, excluding many pediatric patients with moderate to severe disease.41 It is important to consider how future trials will be developed to allow for greater enrollment of pediatric patients and better capture of the dynamic course of HS.

Pediatricians should be aware of the wide range of comorbidities associated with HSped. Estimated comorbidity rates in HSped range between 34% and 93%.10,44,45 Although there are no validated guidelines for screening for comorbidities in HSped, there are some recommendations.34,44,46

> Metabolic

Obesity is the strongest association with HSped, with estimated obesity rates in patients with HSped between 32.5% and 68.7%.10,16,38,42,44,48–51 This risk was elevated regardless sex, age or race. However, many studies on HSped examined BMI according to adult measurements and not percentiles, potentially confounding the true rate of obesity in this population. Despite the association, there is minimal evidence showing that weight reduction improves disease severity.

Although the metabolic syndrome is not well defined in childhood, patients with HSped are at risk for several of the individual diagnoses that define the syndrome.52 A retrospective case-control study of 153 patients with HSped showed a 12-fold higher prevalence of metabolic syndrome compared to controls.45 Higher rates of hyperlipidemia are observed in HSped (3.2% to 16%), although lower than that reported in adults.10,16,44,49 Acanthosis nigricans, often a marker of hyperinsulinemia, was found in 17 of 73 patients with HSped in one study.44

Studies differ as to whether patients with HSped have increased rates of high blood pressure.10,16,38,44,45,49,50,53

> Psychiatric

In a retrospective study of 49,280 adults and 3042 children with HS, the percentage of children with a new diagnosis of depression (14%) was lower than that of adults (16.3%). However, the hazard ratio when population rates were compared by age was higher for HSped (1.87) than for adult HS (1.61).54 Another study of 25 adolescent HS patients found screening PHQ-2 positive depression in 32% of patients, more than double the rate seen in a general adolescent population.55

Anxiety and other psychiatric disorders also increase in HSped. In a cross-sectional study of 87,053,155 US hospitalizations, HS accounted for 24,666 hospitalizations (899 HSped), and was associated with a primary or secondary diagnosis of mental health disorder (odds ratio 2.53).56 Estimated rates of these additional conditions vary from 0% to 33.6% and change depending on sample size, country, and study design.10,16,44,45,57

In a cohort of 153 patients with HSped, the rate of psychiatric comorbidity increased from 15.7% to 23.5% in 5 years, which was not seen with somatic comorbidities, highlighting the increased risk of developing psychiatric disorders after a diagnosis of HS.45 Another study of 16,489 adults and children with HS also showed an increase in mood disorders after diagnosis.53 Interestingly, in a study of 195 children from an obesity clinic, having a psychiatric comorbidity was associated with a diagnosis of HSped.58

> Endocrinological

Recent studies have shown that prepubertal onset of HSped is more common than previously thought, with prevalence estimates ranging from 7.7% to 42.4%.20,21,39,42,59 It was previously thought that HSped was associated with precocious puberty or premature adrenarche based on case reports.32,60–62 Although cases of precocious puberty and premature adrenarche are observed, they represent <5% of patients in multiple large HSped studies.10,16 ,21,40,44,45

Polycystic ovary syndrome ( PCOS) is seen more frequently in HSped, with reported incidences in women ranging from 0% to 17.5%.16,21,40,42,44,45 In a multidisciplinary clinical study of PCOS, 14 (15.2%) of 92 patients had concomitant HS.63 Rates of hirsutism (6.8% to 19.7%) and irregular menses (6.5% to 25.4%) may be even more common .40,42,44 Screening for signs of PCOS and precocious puberty is recommended, but in the absence of these, hormonal analysis is not indicated.44,47

Diabetes seems to be more common in HSped . A study of 73 patients with HSped showed elevated rates of prediabetes (6.8%) and diabetes (type 1 and 2 combined, 2.7%), with men being more likely to have prediabetes than women (21% and 3 %, respectively).44 Another study of 58 patients in Asia found 17.2% diabetes, with no differences between the onset of pediatric and adult HS.50 However, another study of 153 patients with HSped showed no differences in rates of type 1 diabetes compared to controls, nor cases of type 2 diabetes.45

> Cutaneous

Acne, dissecting cellulitis, and pilonidal cysts can occur along with HS in an association called follicular occlusion tetrad.64

Acne (13.7% to 68.8%), psoriasis (2.1% to 11%), and pyoderma gangrenosum (0.2% to 0.6%) are more common in pediatric patients with HS compared to others. children.10,16,38,40,42,44,45 Severe HSped is more likely to be associated with pilonidal cysts (4.1% to 16.4%).16,40,44 Patients should be seen by a dermatologist at least once a year to check for related skin conditions.47

> Down syndrome and other genetic conditions

Down syndrome or trisomy 21 is strongly associated with HS. Between 2% and 14% of patients with HSped have Down syndrome, and these patients have a younger age of onset of HS.10,12,40,42,44,45,65–67 Patients with Down syndrome have higher overall rates of follicular occlusion disease, such as keratosis pilaris and folliculitis: 44.9% in 1 study of 243 pediatric dermatological patients with Down syndrome, and 38.9% in another study of 203 patients with Down syndrome. this syndrome.67,68 Periodic screening for HS should be performed in all patients with Down syndrome. Partial or complete trisomy 1369-75 and ichthyosis-deafness keratitis syndrome76,77 are also associated with HSped.

> Other comorbidities

Patients with HSped should be screened for inflammatory bowel disease (IBD)34,47 since IBD occurs at higher rates in patients with HSped than in the general pediatric population (0.7% to 3.3%).10, 16,45,78,79 In 1 study of 109 patients with HSped, 48.6% had gastrointestinal symptoms and 11.3% of them were diagnosed with IBD.78 Additional associations include inflammatory arthritis, anemia, atopic dermatitis, and asthma.10,16,40,42,44,45,54

Syndromic HS has also been reported in a subset of patients, typically in association with autoinflammatory disease.80,81 In these cases, HS may occur in conjunction with pyogenic or psoriatic arthritis, ankylosing spondylitis, pyoderma gangrenosum, and acne. These patients may respond to different therapies such as IL-1 blockade.

> Quality of life

HSped has a massive impact on patients’ quality of life (QoL).

Concerns about sports participation, ability to shave, and peer judgment are commonly reported.44 A quality of life study of 25 patients with HSped showed an average Skindex-Teen score of 45.7 (maximum 84, range 0–84), dramatically worse compared to patients with psoriasis (21.1) and atopic dermatitis (29.4).55,82

The most impactful factors were the appearance of the affected skin and feelings of frustration about HS. The quality of life worsened during Hurley’s time. The Family Dermatology Quality of Life Index was comparable to other inflammatory skin disorders, with the most impactful factors being time spent caring for your child with HS and personal emotional distress related to your child’s condition.55

There are currently no formal published clinical studies that include children or adolescents. Management of HSped is extrapolated from treatment patterns in adults, and is based on clinical presentation, type and location of injury, and severity.

Overall HS treatment data are limited by small sample sizes, inconsistent outcome measures, and lack of validated HSped measurements. This section is guided by the 2019 American Clinical Treatment Guidelines for Hidradenitis Suppurativa and recent publications, and supported by pediatric case series and case reports.83

> Pain management

HS lesions can be quite painful. The use of Paracetamol or Ibuprofen should be recommended when patients are in flare, with prudent use of opiates in exceptional cases. Patients may need to be excused from physical activity during flares.

> Wound care

Abscesses and sinus tracts are often associated with serous drainage, purulent drainage, and malodor, and can severely affect quality of life. Therefore, adequate wound care education and adequate, cost-effective supplies for wound management are required, keeping in mind that insurance coverage may be variable. Patients may benefit from absorbent foam, hydrogel, and non-adherent gelling fiber dressings.84

> Treatments

Topical antimicrobial agents

Washes with benzoyl peroxide, chlorhexidine, and zinc pyrithione are commonly used to clean HS affected areas daily to decrease microbial colonization. Potential tissue irritation and discoloration are common adverse effects of benzoyl peroxide. Clindamycin 1% solution applied once or twice daily is used as a first-line treatment for mild to moderate HS.85 It is often combined with benzoyl peroxide washes to minimize bacterial resistance. Alternatively, cream with 15% resorcinol can be used daily for its antiseptic and keratolytic properties.40,86 Irritation and burning are possible side effects. Topical dapsone gel is supported only by expert opinion, as clinical data are lacking.83

Oral antibiotics

For HS flares, systemic antibiotic therapy is indicated.

Tetracyclines are used as first-line antibiotics due to their broad antibacterial coverage and anti-inflammatory action. Doxycycline is commonly used for mild to moderate HS over a 12-week course or as long-term maintenance if necessary.83

Patients should be counseled about the common side effects of photosensitivity, gastrointestinal upset, and hepatotoxicity in rare cases. Alternatively, minocycline may be used in children ≥ 12 years of age. Possible adverse effects include dizziness, blue discoloration of the skin or gums, and hepatotoxicity or drug hypersensitivity reactions in rare circumstances.

Rifampicin is recommended in combination with clindamycin for 8 to 12 weeks in patients who do not respond to treatment with tetracyclines.83 15% of patients experience nonspecific gastrointestinal discomfort; however, the risk of Clostridium difficile colitis must be considered . Systemic dapsone, a sulfone with anti-inflammatory properties, has been used as a second-line treatment in adults with refractory HS, with retrospective case series reporting 60% of patients with clinical improvement.87,88

Intravenous antibiotics

Based on limited case series, ertapenem, a broad-spectrum carbapenem antibiotic, is effective for severe flares in adult patients with HS.83,89

Intralesional corticosteroids

Intralesional injection of triamcinolone acetonide has demonstrated efficacy for the treatment of acute nodular HS lesions and can be used in willing pediatric patients.90 The addition of 1% lidocaine injection may provide temporary anesthesia. Age-appropriate distraction techniques and topical lidocaine cream 30 minutes before the injection may make the procedure more tolerable.

Biological therapy

A systematic review of the use of biologics in HSped found that the mean disease duration before initiating biologic treatment was 3.5 (±2.9) years.91 The majority of patients were Hurley stage 2. In the pooled analysis of 26 HSped cases treated with biologics, 23.1% experienced complete resolution, 73.1% had partial response, and 3.8% had no improvement.91

Adalimumab is a TNF-α inhibitor approved for use in patients ≥ 12 years of age with moderate to severe HS. Its approval in adolescents was based on adult studies with no additional clinical trial data.92 Dosing is based on body weight. Other TNF-α inhibitors can be used for refractory HSped, including infliximab, a chimeric monoclonal antibody infusion. However, data on the use of etanercept in HS are mainly unsupported.93–95

Adverse effects of TNF-α inhibitors include an increased risk of opportunistic infections and reactivation of tuberculosis and hepatitis B.96 There is a small increased risk of malignancy, particularly lymphomas, which is potentiated by concomitant use of immunosuppressants. Patients on TNF-α inhibitors (especially adalimumab) are at risk of autoimmunity with formation of anti-drug antibodies and lupus-like reactions. Paradoxical exacerbation of HS has been reported with the use of TNF-α inhibitors.97

IL-17a inhibitors (secukinumab, ixekizumab), approved for pediatric psoriasis, have been effective in limited case series of HS in adults.98,99 A systematic review of the use of biologics in HSped reported limited cases of treatment with ustekinumab (IL-12/23 inhibitor) and anakinra (IL-1 inhibitor).91

All patients should be tested for tuberculosis and hepatitis B before starting treatment with a biological agent. All age-appropriate vaccines should be administered 1 month before starting biological products, and live vaccines should not be administered to children receiving these products.100

Hormonal therapies

Hormonal therapies are used with increasing frequency in adolescents with HS; however, data are limited.101 Combined oral contraceptives are used as adjunctive therapy in women with HS. Progestins with greater antiandrogenic activity are recommended (i.e., norgestimate, drospirenone). Data suggest that patients with perimenstrual flares of HS and those with concomitant treatments respond better than patients without cyclic flares or on combined oral contraceptive monotherapy.102 Before starting a combined oral contraceptive, patients should be adequately screened for risk of thrombosis and stroke, hypertension, migraine with aura and liver tumor. It should be noted that rifampicin will decrease the effectiveness of combined oral contraceptives.103

Spironolactone , an antiandrogen and potassium-sparing diuretic, can be used alone or together with a combined oral contraceptive in postmenarcheal women with HS. Side effects include menstrual irregularity and breast tenderness. Hyperkalemia may occur, mainly in those with renal dysfunction; however, serum potassium monitoring is not required in otherwise healthy people.104

Finasteride , a systemic 5a-reductase inhibitor, has been used in adults and children with HS who do not respond or are intolerant to other antiandrogens.105 Potential side effects include headaches, dizziness, nausea, breast tenderness, gynecomastia, and irregularity. menstrual. Metformin decreases hepatic glucose production and is an antiandrogen. It is an adjunctive therapy used predominantly in patients with HS and associated hyperinsulinemia.106 Potential side effects include gastrointestinal upset, decreased absorption of vitamin B12, and lactic acidosis.

Retinoids

Isotretinoin has demonstrated variable efficacy in limited retrospective and prospective HS studies.107-109 The typical dose is 0.5 to 1.0 mg/kg per day . Due to its teratogenicity, isotretinoin is subject to a mandatory risk management program by the US Food and Drug Administration for all patients. Acitretin, also a teratogen, should be used with extreme caution in patients of childbearing potential due to its long half-life. Other side effects of retinoids include cheilitis, hyperlipidemia, transaminitis, and rarely pancreatitis. Due to the risk of pseudotumor cerebri, isotretinoin should not be used with systemic tetracyclines.

Systemic immunomodulators

Systemic corticosteroids at 0.5 to 1.0 mg/kg per day for 7 to 10 days are used to control HS flares and may serve as a bridge to other systemic therapies.83

Combination therapy using cyclosporine with adalimumab has been reported in adult patients with severe HS.110 Side effects of cyclosporine include immunosuppression, kidney damage, hypertension, and decreased cell counts; therefore, close clinical and laboratory monitoring is required. Based on adult case series, colchicine at 0.5 mg twice daily is used as adjunctive therapy most commonly with systemic tetracyclines.111 Methotrexate and azathioprine are not recommended for HS.83

Surgical Management

When recurrent HS lesions form scars and sinus tracts, surgical intervention may be necessary.

Incision and drainage should only be performed in acute and painful disease with very purulent abscesses. It is not recommended as definitive therapy due to high recurrence rates. Other surgical techniques, such as puncture debridement (for small nodules) or tissue-sparing unroofing (for epithelialized tracts), can be performed in both the acute and chronic settings with careful attention to pain control.112

Larger areas of severe, chronic disease may require extensive local excision with the possible need for skin grafts or flaps. Depending on the age of the patient and the extent of the surgical procedure chosen, these may be performed under local or general anesthesia by a pediatric, plastic or dermatological surgeon.

Laser hair removal using long pulse neodymium-doped yttrium aluminum alexandrite or garnet lasers may be helpful in patients with mild to moderate HS. Multiple treatments are required, are moderately painful, and may not be covered by insurance, so appropriate patient selection is necessary. In a prospective study of 20 adults with mild to moderate HS treated with alexandrite laser compared to 20 untreated control patients, treated patients reported decreased pain, improvement in quality of life metrics, and longer flare-free periods. at 15 and 30 weeks.113

Botulinum toxin A injections to reduce sweating in the armpits and groin have been reported to be beneficial in small case series and case reports in both pediatric and adult patients with HS, particularly in those with associated hyperhidrosis.114

> Patient education

HS is a chronic disease with long-term health implications. Educating patients and their parents about the disease is vital to empower them and increase adherence to treatment.

HSped is an underrecognized inflammatory disease with tremendous impact on quality of life during an important time of development. With a high burden of comorbidities and multiple treatment options, multimodal management with medications, surgery, and multiple subspecialists is often necessary for more advanced diseases. Early recognition and intervention will allow doctors to act during a window of opportunity where treatments are most effective.

Hidradenitis suppurativa is a chronic, inflammatory, and debilitating disease that presents as painful nodules and abscesses with a predilection for skin folds. Its early identification can be a challenge, since it depends on patients presenting typical skin lesions with recurrent outbreaks of the disease, and the diagnosis can often be delayed or confused with infection.

There are various therapeutic options depending on the severity of the disease, although management is usually multimodal. It is important to increase awareness about HS in primary and emergency care settings to achieve early diagnosis, and educate patients to improve treatment adherence and quality of life.