Diagnosing and Managing Scarring Alopecia in Adolescents

Since pediatric alopecia is a rare condition in children, there are minimal studies of institution experiences and their methods for the hair loss treatment. | Image Credit: littlewolf1989 - stock.adobe.com

The diagnosis and treatment of scarring alopecia, specifically among adolescent patients, was the focus of a session at the Society for Pediatric Dermatology Annual Meeting held in Toronto, Ontario, Canada from July 11 to July 14, 2024.

The session was presented by Marissa Joseph, MD, MSc, FRCPC, clinician teacher at the University of Toronto, medical director at Women’s College Hospital, and staff physician at the Hospital for Sick Children. Joseph highlighted cases encountered in her practice to illustrate the recommended approach to managing scarring inflammatory scalp dermatosis in children and adolescents. Her presentation emphasized the importance of trichoscopy and histopathology in the evaluation of scarring alopecia.

Some types of scarring alopecia recognized during the session were central centrifugal cicatricial alopecia (CCCA), discoid lupus erythematosus, and lichen planopilaris (LPP). Symptoms for these subtypes include scaling, stinging, burning, itching, erythematous annular plaques, scalp pruritus, lander papules or pustules, and in some cases patients are asymptomatic. Regions of hair loss can include the vertex, frontal, parietal or patchy distribution, confluent random plaques, primarily frontotemporal involvement, or could vary.

While primary and secondary scarring alopecia symptoms are similar in pediatric patients and adults, pediatric alopecia is more rare. Since it is a rare condition in children, there are minimal studies of institution experiences and methods for treating this type of hair loss.

"The scarring alopecia and the loss of follicles and the permanence of that disfigurement really belies the need to have better diagnostics and therapeutics for this patient population,” Joseph said.

On average, patients with alopecia reach stabilization after 2 years. Common alopecia diagnoses include folliculitis decalvans, LPP, aplasia cutis congenital, tinea capitis, morphea, discoid lupus erythematosus, dissecting cellulitis, keratosis follicularis spinulosa decalvans (KFSD), and pachyonychia congenital.

Joseph referenced a case in her practice that included a 16-year-old female Black patient with progressive itching and associated hair loss on the vertex of her scalp. The patient reported tenderness when her hair was braided and worn in tight cornrows. Her original physician suggested hair washing daily and an anti-yeast shampoo, but the patient did not adhere to this treatment because she did not wash her hair frequently, which is common for individuals with coily curly hair.

After Joseph reassessed this case, it appeared the patient had CCCA, a progressive, inflammatory scarring alopecia, which is the most common form of primary scarring alopecia among Black women in South Africa (2.7%) and Black women in the US (5.6%).¹ Oftentimes, CCCA occurs in the absence of clinical signs of overt inflammation, like redness and scaling, and instead, fibrosis is the predominant feature.

An important tool for an alopecia diagnosis and biopsy is trichoscopy, Joseph said. For patients with CCCA, a trichoscopy reveals hair loss beginning with the central scalp that gradually spreads outward in a centrifugal pattern. Patients may experience itching, tenderness, a burning sensation, and hair breakage in the affected areas. Hair breakage also commonly occurs in earlier diagnosis of CCCA.

Hair loss in patients with alopecia who have coily curly textured hair does not typically fit into the pattern of alopecia areata. This usually results in "premature diagnostic closure," which Joseph described as a cognitive error in research that occurs when the clinician makes a quick diagnosis and stops data collection prior to considering other possibilities.

Typically, patients with coily curly hair are diagnosed with traction alopecia because their hair type does not fit into the pattern of alopecia areata and physicians assume the hair loss is from tension-forming hair practices. Historically, physicians referred to CCCA as “hot comb alopecia," a popular tool that individuals with coily curly hair use.

However, research has found a mutation in the gene that is involved in lipid metabolism and hair shaft formation known as peptidyl arginine deiminase type III (PADI3), known for translationally modifying other proteins important for the rest of the hair shaft, associated with CCCA.¹

The session highlighted the importance of non-clinical factors related to CCCA. This includes research showing that even non-obese women with a confirmed CCCA diagnosis have a 3 times greater risk of developing diabetes. Additionally, there seems to be a difference in breast cancer prevalence among women with CCCA.¹

Joseph referred to a study of 6 children between 14 years old to 19 years old with biopsy-proven CCCA.² The group of children discussed hair care practices and risk factors, but 5 out of the 6 patients did not use relaxers and wore their natural hair without adding significant tension to their scalp. The majority (5 of 6) of the pediatric population reported tender scalp and had a known family history of CCCA. Hair loss occurred primarily on the vertex of the scalp in 4 of the 6 patients. Every patient had concentric perifollicular fibrosis on their histopathology.

In terms of management and approach to treatment, diagnosis should first be established by a clinical exam like a dermoscopy or biopsy, Joseph explained. Patients should also be provided with education on recommended hair care practices. Treatment of CCCA can be divided into 2 stages: an anti-inflammatory phase and an adjuvant hair regrowth phase. During the anti-inflammatory phase of treatment, common medications include topical corticosteroids, injections, and oral medications.

Some forms of management for CCCA, specifically Black women with CCCA, include hair camouflage.³ Hair camouflaging techniques can ensure effective concealment while improving well-being and minimizing alopecia aggravation. In some cases, braiding in alternate directions is suggested to reduce tension and avoid hair follicle damage. Other forms of hair camouflage include sewn-in cranial prostheses, hair extensions, topical hair fibers, scalp micropigmentation, microblading, and in severe cases, hair transplantation.

Joseph described another case of a 9-year-old boy who experienced progressive hair loss, primarily on the crown of his head, and after a biopsy, was diagnosed with scarring alopecia. Interestingly, the patient also had erythema of the eyebrows and extensive follicular hyperkeratotic papules on his lateral arms, thighs, chest, and back. Additionally, there was a slight thickening xerosis of the palms. Ultimately, the patient was diagnosed with CCCA and KFSD.

The management for this case began with an ophthalmologic assessment, followed by a high potency topical steroid, which resulted in a minimal response. Treatment then shifted to isotretinoin (0.5 mg/kg/day) with minimal inflammation visible. After 5 months of therapy, the patient did not have any improvement in keratosis, but there was no progression of the disease.

Joseph concluded the session by emphasizing the key takeaways when approaching and managing pediatric alopecia. She explained that trichoscopy and biopsy are necessary to diagnose scarring alopecia, and physicians must learn to pull away from premature diagnostic closure. Recognizing symptoms of alopecia can assist with mitigating progression and further disfigurement, especially in scarring alopecia, which is a challenging condition to treat and typically requires multiple therapies because there is no known cure.

References

1. Malki L, Sarig O, Romano MT, et al. Variant PADI3 in central centrifugal cicatricial alopecia. New England Journal of Medicine. 2019;380(9):833-841. doi:10.1056/nejmoa1816614

2. Eginli AN, Dlova NC, McMichael A. Central Centrifugal Cicatricial Alopecia in Children: A Case Series and Review of the Literature. Pediatr Dermatol. 2017;34(2):133-137. doi:10.1111/pde.13046

3. Santoro C. Evaluating hair camouflage as a tool for black women with alopecia: A critical review. The American Journal of Managed Care®. January 18, 2024. Accessed July 18, 2024. https://www.ajmc.com/view/evaluating-hair-camouflage-as-a-tool-for-black-women-with-alopecia-a-critical-review