Diagnostic Criteria for Hidradenitis Suppurativa May Overlook Pediatric Patients

Pediatric patients with hidradenitis suppurativa (HS) may experience disease recurrence less frequently than required by existing diagnostic criteria, according to study findings published in JAMA Dermatology.

HS is a disfiguring and debilitating chronic inflammatory skin disease associated with considerable diagnostic delay, ranging from a mean duration of 2 to 7 years.

Consensus-derived diagnostic criteria for HS exist to guide accurate clinical diagnosis, which has shown applicability for adult patients, noted researchers. But these criteria have not been evaluated in pediatric patients, despite most patients reporting onset of HS during adolescence.

“Consequences of diagnostic delay for patients with HS of all ages include possible disease progression and delayed medical and procedural interventions. In addition, diagnostic delay hinders screening and treatment of HS-associated comorbidities and functional and social impairment,” said the study authors.

In seeking to assess the utility of existing HS diagnostic criteria in pediatric patients, they conducted a retrospective, cross-sectional study of data derived via electronic medical records from 2 sites of a single academic tertiary care center. The analysis examined the proportion of physician-diagnosed pediatric patients with HS who met diagnostic criteria, and secondary objectives included demographics, disease characteristics, and diagnostic patterns among these patients.

Eligible patients were those born after January 1, 1993, and assigned International Classification of Diseases, Ninth and Tenth Revisions (ICD-9/10) codes for HS (ICD-9 705.83/ICD-10 L73.2) between January 1, 2012, and July 1, 2021. Patients 18 years or older at diagnosis and those with inaccessible diagnostic visit notes or unintentionally assigned HS ICD-9/10 codes were excluded.

Documentation from the diagnostic visit, defined by the first-recorded HS ICD-9/10 code, was reviewed to determine the diagnosing physician’s specialty, patient characteristics, and fulfillment of all 3 major HS diagnostic criteria required for diagnosis: typical lesion, typical location, and recurrence (2 or more lesions within 6 months).

Medical chart review was conducted from November 22, 2021, to January 12, 2022, and the analysis was conducted from January 12, 2022, to January 15, 2022.

A total of 297 adolescents with HS were included in the study (median [IQR] age at diagnosis, 14.0 [13.0-16.0] years), of which 123 patients were female (78.1%), 78 self-identified as Black (26.3%), and 116 self-identified as Hispanic (39.1%).

Of the patients with documented Hurley staging (n = 73), 39 (53.4%) had moderate or severe disease at diagnosis. Oral antibiotics were the most common treatment prior to diagnosis (31.0%), but 69 (23.2%) patients were never treated and no patients received biologics.

Documentation from the diagnostic visit indicated that major diagnostic criteria were fulfilled in 170 (57.2%) patients and 127 (42.8%) patients did not meet all 3 major HS diagnostic criteria. Of those who did not fulfill HS diagnostic criteria, 122 (96.1%) did not meet the recurrence interval criterion (2 or more lesions within 6 months), followed by 4 patients who did not have a typical lesion and 3 who did not have a typical affected location.

Ninety-six patients with complete medical records who did not meet the recurrence interval criterion had documentation from additional visits in the health system. From these patients, 59 (61.5%) had documentation of 1 or more additional lesions consistent with HS.

Further review of these additional records showed that 26 of these 59 (44.1%) patients met the recurrence interval criterion after diagnosis and 44 (74.6%) had recurrent lesions within a 1-year interval (median [IQR], 6.5 [3.5-12.2] months).

Patients who received their diagnosis from a pediatric dermatologist were more likely to have documented fulfillment of all major diagnostic criteria compared with those who received their diagnosis from nondermatologists (86 [67.7%] of 127 vs 84 [49.4%] of 170; P = .002).

“Because pediatric patients with HS present to various specialties, increasing awareness of HS beyond the dermatology setting is key to minimizing diagnostic delay and delayed therapy,” said the study authors.

They concluded that future prospective studies are warranted to determine if revisions to the HS diagnostic criteria are needed to facilitate timely HS diagnosis in a pediatric population.

Reference

Kittler NW, Williams JC, Kudlinski MV, et al. Evaluation of hidradenitis suppurativa diagnostic criteria in pediatric patients. JAMA Dermatol. Published online October 26, 2022. doi:10.1001/jamadermatol.2022.4564