Diagnostic Delays in Children With Hidradenitis Suppurativa, Irreversible Damage

Many children with hidradenitis suppurativa (HS) present with comorbidities and advanced disease at time of diagnosis, reflecting a considerable gap in the clinical recognition and diagnosis of HS, a disorder that is currently poorly characterized in the pediatric population. This is according to a study published in JAMA Dermatology.

The cross-sectional study was a retrospective medical record review of 481 pediatric patients with HS from 10 dermatology clinic clinics in the United States, Canada, Australia, Italy, and Israel. Only medical record data recorded from 1996 to 2017 were included in this review. The investigators examined patient demographics, disease severity, clinical features, comorbidities, and treatments in this population.

The referring diagnosis was HS in 39% of patients. Other common referring diagnoses were cysts/abscesses in 27%, acne in 12%, growth/bumps in 9%, folliculitis/furunculosis in 8%, and unknown diagnosis in 8% of patients. Pediatricians were the most common clinicians who referred patients (45%).


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Approximately 80% (n=386) of the patients were girls. At disease onset, the mean age was 12.5 years. The average age at time of diagnosis was 14.4 years. Up to 41% (n=111) of patients had a family history of HS. The most common initial signs/symptoms at disease onset included cyst/abscess (48%), pain/tenderness (25%), and papules/pustules (24%).

Evidence of skin scarring was observed in 48% (n=233) of patients at time of the initial dermatologic assessment. This finding indicates that a substantial percentage of children with HS experience a delay in diagnosis, the investigators believe. .

Nearly half (47%) of patients were in Hurley stage 1, defined as the presence of abscesses, no sinus tracts, and no cicatrization. In addition, approximately 45% were at Hurley stage 2 (recurrent abscesses with tract formation and cicatrization) and 8% of patients were at Hurley stage 3 (diffuse or near-diffuse involvement/multiple interconnected tracts and abscess).

Up to 88% of patients received topical treatment for HS, including clindamycin (75%) and benzoyl peroxide (60%). In addition, 79% of patients received systemic antibiotics. Emergency department visits due to HS were reported in 22% of patients, and 8% of patients were hospitalized because of their condition.

The majority of patients (79%) had complications associated with their disease, including scarring and contractures (80%).Also, most patients (85%) had comorbid conditions at presentation. The most common comorbidities were obesity (65%) and acne vulgaris (29%).

Limitations of the study included its cross-sectional and retrospective design as well as the lack of data regarding disease severity categories for some patients.

In an accompanying editorial, Joslyn S. Kirby, MD, and colleagues suggest that there currently exists a need to increase recognition of HS “by health care clinicians and decrease delays in diagnosis and treatment through advocacy, education, and research.”

Dr. Kirby and colleagues suggest that HS is more common than previously thought, and there appears to be a trend toward earlier presentation in adolescence and childhood. Despite the potential increase in case numbers, HS remains largely “unseen” in clinical practice, and HS data specific to children are lacking. Likewise, the true prevalence of the disease in children is possibly greater than currently reported, given the familiarity gap among pediatricians.

As discovered in this new study, girls are more frequently affected by HS. Previous reports suggest children of color are also disproportionally diagnosed with HS, indicating the disparities inherent in the disease. Children of color may be particularly vulnerable to the effects of HS, as these patients may be more likely to have limited access to high-quality care and clinical trials.

Dr. Kirby and colleagues added that quality of life represents an important metric in the prognosis of children with HS, as reports show the disease in children affects this metric twice as that of psoriasis. Rates of depression also appear to be higher in children with HS than that of the general adolescent population. The disease also has negative effects on family members. These findings further demonstrate the importance of early recognition and diagnosis, the researchers purport.

Improvements in all HS-related domains may only be realized with optimal management, however, emphasizing the need for better treatment options specific to this pediatric population. “Without formal clinical testing and drug approval for pediatric patients with HS, children will be denied access to effective and state-of-the-art care,” Dr. Kirby and colleagues wrote.

They added that pediatric patients with HS “should be involved in the development of core outcome sets, treatment goal setting, and advocacy for themselves” to improve the trajectory of care outcomes.

Disclosure: This clinical trial was supported by AbbVie. Several study authors declared affiliations with biotech, pharmaceutical, and/or device companies. Please see the original reference for a full list of authors’ disclosures.

References

1. Liy-Wong C, Kim M, Kirkorian AY, et al. Hidradenitis suppurativa in the pediatric population: An international, multicenter, retrospective, cross-sectional study of 481 pediatric patients. JAMA Dermatol. Published online February 24, 2021.  doi:10.1001/jamadermatol.2020.5435

2. Kirby JS, Zaenglein AL. Recognizing the effects and disparities of pediatric hidradenitis suppurativa. JAMA Dermatol.  Published online February 24, 2021. doi:10.1001/jamadermatol.2020.5434

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