Paediatric scarring alopecia: A retrospective analysis from India

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Childhood alopecia has a significant psychological impact, impairing growth and development. Although non-scarring alopecia contributes majority of cases (90%), scarring alopecia is also seen, albeit rarely, in children. Data regarding clinico-demographic, histopathologic parameters and treatment response in paediatric scarring alopecia remain limited with most data originating from the western world. We describe a retrospective record review of paediatric scarring alopecia from a tertiary care institution in India.

Of 3000 records spanning various childhood dermatoses over the last five years (January 2020 to December 2024), we identified 15 cases of childhood scarring alopecia. Scarring alopecia was defined as permanent hair loss due to irreversible follicular damage and replacement by fibrous tissue, confirmed clinically and histopathologically. The mean (SD) age of affected children was 10.4(2.9) years with a range of 6-16 years. The most common age group affected was school-going children (n=9) (6-10 years), followed by adolescents (n=6). The male-to-female ratio observed was 1:2. The mean (range) duration of illness before presentation was 14.4 (3-36) months. Lesional pruritus and pain symptoms were observed in 4/15 (26.7%) and 5/15 (33.3%) cases, respectively. The most common locations of alopecia, in order of frequency, were the vertex (n=7), diffuse involvement (n=3), parietal area (n=3), occiput (n=1) and frontal region (n=1). The maximum diameter of the alopecic patches ranged from 2-7 cm with a mean diameter of 3.77 cm. On clinical examination and trichoscopy, the most common findings were scarring (n=14, 93.3%), loss of follicular ostia (n=14, 93.3%), erythema (n=6, 40%), perifollicular scales (n=6, 40%), pustules (n=3, 20%), tufting of hair (n=1, 6.7%), follicular plugging (n=1, 6.7%). Trichoscopic examination was performed using a Dermlite DL-4 in polarised mode at 10x magnification to evaluate scalp and follicular features.

In our cohort, distinct trichoscopic features were observed for each primary cicatricial alopecia subtype. Lichen planopilaris (LPP) characteristically showed perifollicular scaling and perifollicular erythema [Figure 1a]. Pseudopelade of Brocq displayed ivory white areas with loss of follicular openings [Figure 1b]. Morphoea-associated alopecia exhibited shiny, structureless white areas and decreased follicular density. Chronic cutaneous lupus erythematosus (CCLE) showed thick follicular plugs and telangiectatic vessels. Folliculitis decalvans demonstrated tufting of hair and yellowish perifollicular pustules [Figure 1c]. These consistent trichoscopic patterns aid in differential diagnosis and early recognition of disease subtype. The distribution extended to non-scalp hair in one patient with involvement of eyebrows. Regarding nail involvement, regular coarse pitting was observed in one patient. The quality of life assessed by mean (SD) children’s dermatology life quality index (CLDQI) was 7.4 (2.3) with a moderate effect on quality of life (QOL). Overall, primary cicatricial alopecias (PCA) (n=13, 86.7%) were more common than secondary cicatricial alopecia (SCA) (n=2, 13.3%). Amongst the PCA, the most common diagnoses in decreasing order included lichen planopilaris (LPP) (n=6, 40%), pseudopelade (n=3, 20%), morphoea (n=2, 13.3%) [Figure 1d], chronic cutaneous lupus erythematosus (n=1, 6.7%) and folliculitis decalvans (n=1, 6.7%). Kerion (n=1, 6.7%) [Figure 1e] and post-traumatic scar (n=1, 6.7%) were the only SCA observed. LPP and CCLE were distinguished based on clinical and trichoscopic features. LPP typically presents with perifollicular erythema and violaceous hyperkeratosis, while CCLE shows follicular plugging with atrophic depigmented plaques. Trichoscopy revealed perifollicular scaling and blue-grey dots in LPP [Figure 2a], whereas CCLE demonstrated thick arborising vessels and prominent follicular plugs [Figure 2b]. Biopsies were performed in all cases using vertical sections from the active margin of lesions, guided by trichoscopic findings to optimise diagnostic yield.

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Figure 1a: Multifocal scarring alopecia in an adolescent, depicting perifollicular erythema and violaceous hyperkeratosis, consistent with lichen planopilaris.

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Figure 1b: Irregular smooth atrophic alopecic patches without any signs of inflammation or scaling suggestive of pseudopelade of Brocq.

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Figure 1c: Multiple cicatricial patches over scalp in a child with yellow follicular pustules and tufts of hair suggestive of folliculitis decalvans.

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Figure 1d: Atrophic indurated hyperpigmented plaque over neck extending towards occiput with loss of hair suggestive of morphea.

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Figure 1e: Boggy dome shaped oedematous plaque studded with pustules and yellowishbrown adherent crusting over frontoparietal scalp suggestive of kerion.

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Figure 2a: Trichoscopy of lichen plano-pilaris showing presence of peripilar tubular casts (blue circle) and scales (yellow star) along with perifollicular violaceous hue (green square). (Dermlite DL4, 10x, polarised mode).

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Figure 2b: Trichoscopy of chronic cutaneous lupus erythematosus of scalp showing areas of scarring with linear telangiectatic vessels (blue star), follicular plugs (blue circle) and adherent keratotic scales. (Dermlite DL4, 10x, polarised mode).

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Histopathology was done in all cases with the most common findings being perifollicular fibrosis (n=14, 93.3%), lymphocytic infiltrate (n=9, 60%), pigment incontinence (n=7, 46.7%) and neutrophilic infiltrate (n=4, 26.7%) [Figures 2c and 2d]. The most common location of infiltrate was perifollicular (n=12, 80%) followed by both interfollicular and perifollicular (n=3, 20%). The most common topical treatments included topical corticosteroids (n=8, 53.3%), followed by topical antibiotics (n=3, 20%), topical tacrolimus (n=2, 13.3%) and vitamin D analogues (n=2,13.3%). Systemic immunosuppressants were initiated in cases showing active inflammation with progressive hair loss unresponsive to topical therapy. The choice of agent was guided by clinical severity and subtype, with methotrexate preferred for LPP, morphea and pseudopelade following standard paediatric dosing protocols. Other systemic treatments prescribed included oral steroids (dexamethasone minipulse) along with oral methotrexate (n=9, 60%), only steroids, azathioprine, rifampicin and hydroxychloroquine in one patient each. Arrest of disease activity was defined as absence of new lesions for 4 weeks. The mean (range) time for arrest of disease activity observed was 28.27(14-60) days. Table 1 summarises various parameters observed in the study. Supplementary Table 1 summarises various clinical and demographic details of the study cohort.

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Figure 2c: Histopathology suggestive of lichen planopilaris shows perifollicular inflammation (blue star), fibrosis (red circle) and follicular destruction (blue arrow). The overlying epidermis appears unremarkable. (Haematoxylin and eosin, 200x).

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Figure 2d: Histopathology of chronic cutaneous lupus erythematosus of scalp shows interphase change involving the hair follicles and interfollicular epidermis. There is perifollicular inflammation (blue star), fibrosis and pigment incontinence (blue arrow). (Haematoxylin and eosin, 200x).

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Cicatricial alopecia can lead to irreversible loss of hair with negative impact on quality of life. It has been broadly categorised as PCA or SCA. PCA was the most common diagnosis in our cohort [Figure 3a], consistent with previous literature.^1,2 The predominance of PCA over SCA in our cohort underscores the importance of early autoimmune evaluation and surveillance in children presenting with progressive scarring alopecia. In PCA, LPP was the single most prevalent entity observed. We observed an overall female preponderance (male:female ratio- 1:2) consistent with previous studies,^1-3 although in LPP, a male preponderance (male:female ratio- 2:1) was seen. A delay in presentation by about 14 months was consistent with previous studies where 79% presented after 1 year.¹ Other findings like vertex location, presence of symptoms like itching and pain and loss of follicular ostia being the commonest findings were also observed in previous cohorts.^1,2 Additionally, we observed findings like erythema, pustules, tufting of hair, perifollicular scales and follicular plugs. These findings should alert paediatricians and dermatologists to the possible cicatricial alopecia to prevent its progression. Histopathology is also essential for diagnosis, and a good clinicopathologic correlation (CPC) helps in deciding proper management. Trichoscopy serves as a non-invasive surrogate to anticipate underlying histopathologic changes. In our study, loss of follicular ostia on trichoscopy consistently correlated with perifollicular fibrosis on histology (observed in 93.3% of cases), reflecting permanent follicular destruction. Perifollicular scaling and erythema corresponded to perifollicular lymphocytic infiltrate, indicating active perifolliculitis. Follicular plugging on trichoscopy was corroborated by dilated infundibula filled with keratin plugs on histopathology, particularly in CCLE. Similarly, pustules and tufted hair mirrored neutrophilic infiltrates and follicular abscesses in cases like folliculitis decalvans. These correlations reinforce the diagnostic and prognostic value of trichoscopy in paediatric scarring alopecia.

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Figure 3a: Common aetiologies of childhood scarring alopecia observed in our cohort (n=15).

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Topical corticosteroids were the most commonly prescribed therapy. Methotrexate along with oral minipulse dexamethasone was the commonest systemic modality used for LPP, morphea and pseudopelade. Methotrexate is considered as the second-line treatment for LPP with 67% efficacy and helps in stabilising disease activity.^3,4 The treatment was well-tolerated at a dose of 0.3-0.5mg/kg/week without major adverse effects and a mean time of stabilisation of about 28 days.⁵

To conclude, our cohort is the only one from Indian subcontinent summarising various epidemiologic parameters of paediatric scarring alopecia. These findings could help determine disease patterns and early recognition of disease to prevent irreversible scarring. However, more prospective studies with a larger sample size are required to corroborate the findings.