Breaking Down Inflammatory Hair Disorders in Children

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Inflammatory hair disorders, particularly scarring alopecias, present a significant challenge in pediatric dermatology. At the 2024 Society for Pediatric Dermatology Annual Meeting in Toronto, ON, Marissa Joseph, MD, from the University of Toronto presented a session titled "Inflammatory Hair Disorders," focusing on the complexities of diagnosing and treating these conditions in children.¹

Understanding Scarring Alopecias

Joseph began by categorizing scarring alopecias into primary, secondary, and congenital types, with a particular emphasis on acquired scarring alopecias. Acquired scarring alopecias are notorious for being difficult to treat, often necessitating off-label and combination therapies. Unfortunately, there is a significant lack of efficacy and safety data for these treatments in pediatric patients.

The Spectrum of Pediatric Scarring Alopecia

Drawing from a Mayo Clinic study,² Joseph highlighted the wide spectrum of pediatric scarring alopecia, underscoring the devastating impact these conditions can have on young patients.

The average time to stabilization of these disorders is approximately 2 years, making early diagnosis and intervention crucial. One of the primary diagnostic tools highlighted was biopsy, which provides critical clarity in identifying the specific type of scarring alopecia.

Case Study: Central Centrifugal Cicatricial Alopecia (CCCA)

Joseph presented a case study involving a young patient initially misdiagnosed with traction alopecia. Upon performing dermoscopy, a peripilar white/gray halo was observed, leading to a diagnosis of central centrifugal cicatricial alopecia (CCCA). CCCA has a prevalence ranging from 2.7% to 16% in Black women and is characterized by fibrosis without the typical signs of redness or inflammation. Biopsy confirmed the diagnosis, with findings aligning with those observed in 94% of patients with CCCA.

A mutation in the PADI3 gene, which is autosomal dominant, is associated with CCCA. Interestingly, non-obese women with biopsied CCCA were found to have a threefold increased risk of diabetes mellitus, highlighting the importance of recognizing and managing this condition beyond the dermatologic implications.

Key Diagnostic and Treatment Insights

Additionally, Joseph emphasized several key points for clinicians treating scarring alopecias in children:

• Trichoscopy and Biopsy: These are essential diagnostic tools that provide critical insights into the nature and extent of scarring alopecias. Early use of these tools can prevent misdiagnosis and facilitate timely intervention.
• Challenging Treatment Landscape: Scarring alopecias require a multifaceted treatment approach, often involving multiple therapies to achieve stabilization and mitigate progression.
• Early Recognition and Treatment: Prompt diagnosis and treatment are vital to prevent significant disfigurement and psychological distress in affected children.

Special Focus: Keratosis Follicularis Spinulosa Decalvans (KFSD)

In addition to CCCA, Joseph discussed keratosis follicularis spinulosa decalvans (KFSD), a rare scarring alopecia. KFSD is associated with photophobia and extensive follicular hyperkeratosis. Management of KFSD includes ophthalmology assessments and the use of systemic retinoids, underscoring the need for a multidisciplinary approach in treating complex hair disorders.

Conclusions

By emphasizing the importance of early recognition, accurate diagnosis through trichoscopy and biopsy, and the need for comprehensive treatment strategies, Joseph's lecture offered a roadmap for clinicians navigating these challenging conditions.

References

1. Joseph M. Inflammatory hair disorders. Presented at the 2024 Society for Pediatric Dermatology Annual Meeting, July 11-14; Toronto, ON.
2. Imhof RL, Cantwell HM, Proffer SL, Tolkachjov SN, Torgerson RR, Tollefson MM. The spectrum of pediatric scarring alopecia: A retrospective review of 27 patients seen at Mayo Clinic. Pediatr Dermatol. 2021;38(3):580-584. doi:10.1111/pde.14543