Bullying in Children With Congenital Ichthyosis

ABSTRACT

Background/Objectives

Bullying of children with chronic disorders is associated with an increased risk of depression, anxiety, poor self‐esteem, and suicidal ideation. Congenital ichthyoses are genodermatoses with extensive visible scaling and inflammation. Bullying is a frequent concern of families with ichthyosis but is rarely discussed or investigated.

Methods

Bullying experience and the impact on children with ichthyosis and their caregivers were investigated utilizing survey methodology and validated questionnaires. Correlations were explored between demographic and ichthyosis characteristics, bullying exposure (Child Adolescent Bullying Scale‐9), child and caregiver psychosocial functioning (Patient‐Reported Outcomes Measurement Information System tools), and family quality of life (Family Dermatology Life Quality Index).

Results

Overall, 49 dyads completed the survey, with a mean child age of 12 years, 43% female, and 35% non‐white. Lamellar (27%), epidermolytic (16%), and with confetti (12%) ichthyosis subtypes were most common. Child‐reported bullying exposure correlated strongly with poor peer relationships (r = −0.63) and moderately with stigma (r = 0.53). Caregiver depression and anxiety scores were moderately correlated with caregiver's perception of child's bullying (r = 0.46 and r = 0.52, respectively). Poor family quality of life (QoL) was moderately correlated with proxy severity, caregiver depression and anxiety, poor peer relationships, and poor mobility.

Conclusions

Recognition of bullying and its potential impact on QoL is an important component of exploring the psychosocial impact of ichthyosis and providing holistic care.

1. Introduction

Bullying is a major public health problem, affecting 20% of school‐age children annually [1]. Cyberbullying is growing as youth access to electronic devices increases. Bullying increases the risk of depression, anxiety, poor self‐esteem, poor school performance, and suicidal ideation, reducing the quality of life (QoL) [2, 3]. Children with common skin disorders experience bullying, especially if lesions are visible [3, 4]. Among children with chronic skin disease, 29.4% of caregivers reported bullying, which correlated with stigma, poor peer relationships, depression, and anxiety [4]. However, research on bullying in children with skin disorders is limited. Most studies are qualitative or based on a single question in the Children's Dermatology Life Quality Index (CDLQI), precluding the acquisition of bullying details [5]. Furthermore, the impact of bullying on children with chronic skin disorders remains underexplored.

Congenital ichthyoses, rare genetic skin conditions characterized by cutaneous scaling and inflammation, generally reduce QoL [6, 7, 8, 9, 10]. Affected children and adults may experience reduced mobility and pain related to thickened skin, fissures, and joint contractures, contributing to increased anxiety and depression [11, 12, 13]. Congenital ichthyoses are often generalized and thus highly visible, predicting a heightened risk of bullying and stigmatization. However, bullying in pediatric ichthyoses has only been mentioned in two primary reports [6, 11], one of which references the single CDLQI question [6]. Bullying is a frequent concern of patients and families and has been discussed at support meetings but is rarely explored at clinic visits or in studies. This project aimed to assess bullying experiences in children with ichthyoses and the potential psychological impact, including on families.

2. Materials and Methods

2.1. Sampling Strategy

English‐speaking children ages 8–17 years with congenital ichthyosis and caregivers (parent/guardian) were included. Caregivers and children ≥ 12 years old provided informed consent and assent, respectively. Both children and caregivers assessed overall severity as mild, moderate, severe, or very severe, and identified affected body parts (Table S1). Gender was child‐reported, but caregivers completed other demographic questions.

2.2. Data Collection

A cross‐sectional survey was conducted from June to September 2022. Subjects were recruited from pediatric dermatology clinics at the Ann and Robert H. Lurie Children's Hospital of Chicago (LCH) and June 2022 Foundation for Ichthyosis and Related Skin Types (FIRST) conference. Both LCH and Northwestern University Institutional Review Boards approved the study. Survey data were housed through Northwestern's Research Electronic Data Capture (REDCap) database. Separate surveys for children (38 questions) and caregivers (34 questions) assessed the child's bullying experience and psychosocial health, family QoL, and caregiver‐reported anxiety and depression. An optional free‐response question allowed participants to elaborate on their bullying experiences.

The Child Adolescent Bullying Scale‐9 Question Version (CABS‐9), a validated bullying assessment scale that is rapid to administer (4–5 min), is the only pediatric scale (≥ 10 years old) focusing solely on bullying victimization and includes appearance‐related bullying [14]. Caregivers also completed the CABS‐9, although a proxy version has not yet been validated.

Children also completed questions from the pediatric Patient‐Reported Outcomes Measurement Information System (PROMIS)‐25 Profile v2.0, assessing mobility, anxiety, depression, fatigue, and peer relationships, and an eight‐question skin‐specific short‐form assessing stigma (PROMIS Pediatric Stigma [PPS]‐Skin) [15, 16]. Caregivers completed adult PROMIS v1.0 short‐forms on self‐reported anxiety and depression and the Family Dermatology Life Quality Index (FDLQI) [15, 17].

2.3. Data Analysis

All analyses were performed using Microsoft Excel. Surveys were scored based on established protocols [14, 16, 17, 18]. Most PROMIS T scores are calibrated to the general population (mean 50, SD 10); ≥ 60 represents moderately increased. As peer relationships and mobility are worse when lower, ≤ 40 is moderately reduced. In contrast, PROMIS Stigma scores are calibrated to a population of children with chronic disease; a T score of 50 represents the mean of children with chronic disease; 40–< 45 suggests mild stigma, 45–< 55 moderate, and ≥ 55% high stigma. CABS‐9, PROMIS/Stigma, and FDLQI scores were compared using Spearman correlation coefficients. Correlations were considered strong (0.60–0.79), moderate (0.40–0.59), or weak (0.20–0.39), with p < 0.05 significant. Analysis of variance (ANOVA) was performed to assess differences in mean bullying and stigma scores by demographic factors (Table 1), with two‐sample unpaired t‐testing if differences were noted. p values from multiple pairwise comparisons were adjusted by the Benjamini–Hochberg method.

TABLE 1.

Demographic characteristics of the congenital ichthyosis cohort (n = 49 dyads).

Characteristics	Number of children	% of total children
Age, years (mean ± SD; median [IQR])	12.0 ± 2.8; median 12 [10–14]
Female gender	20	41
Racial background
Asian/Asian American	11	22.4
Black/African/Afro‐Caribbean/African American	3	6.1
Native American/Alaskan Native	2	4.1
White	32	65.3
Prefer not to answer	1	2.0
Hispanic/Latino origin
Yes	4	8.1
No	44	89.8
Prefer not to answer	1	2.0
Type of school
Public school	40	81.6
Private school	6	12.2
Home school	3	6.1
School setting
Fully in‐person	43	87.8
Home school	3	6.1
Mix of in‐person and virtual/remote	2	4.1
Fully virtual/remote	1	2.0
Average school grades
Mostly As	27	55.1
Mostly Bs	10	20.4
Mostly Cs	8	16.3
Mostly Ds	1	2.0
Other grading scale	3	6.1
Ichthyosis subtype
ARCI—Lamellar ichthyosis	13	26.5
Epidermolytic ichthyosis	8	16.3
Ichthyosis with confetti	6	12.2
Erythrokeratodermias a	5	10.2
ARCI—Congenital ichthyosiform erythroderma	4	8.2
ARCI—Harlequin ichthyosis	4	8.2
Netherton syndrome	3	6.1
X‐linked ichthyosis (XLI)	3	6.1
Keratitis‐ichthyosis‐deafness (KID) syndrome	2	4.1
Ichthyosis follicularis with atrichia and photophobia (IFAP)	1	2.0
Child‐reported severity
Mild	15	30.6
Moderate	25	51.0
Severe/very severe	9	18.3
Proxy‐reported severity
Mild	5	10.2
Moderate	31	63.3
Severe/very severe	13	26.5

Abbreviation: ARCI = autosomal recessive congenital ichthyosis.

^a Erythrokeratodermias includes: erythrokeratodermia variabilis, progressive symmetric erythokeratoderma, and recessive progressive symmetric erythrokeratoderma.

3. Results

3.1. Demographics

Of 64 dyads (children with ichthyosis and a caregiver) who met the criteria, 64 were recruited, of whom 49 dyads completed the full survey (response rate 76.6%) (Table 1). Mean child age was 12.0 ± 2.8 years, with 41% female and 65.3% identifying as white. Ichthyosis subtypes of lamellar (LI; 27%), epidermolytic (EI; 16%), and with confetti (IWC; 12%) were the most common. The majority described severity as moderate (self‐reported 51%, caregiver‐reported 63%) and 70% reported generalized involvement.

Mean child‐ and proxy‐reported bullying exposures were 15 (no/minimal range) and 18 (moderate range), respectively; 67% of children self‐scored as no/minimal bullying (range 9–17) and 33% moderate bullying (range 18–26). Child anxiety and depression scores were each at least moderate in 6.3%; caregiver anxiety and depression scores were at least moderate in 18.4% and 6.3%, respectively (Table 2). Child and caregiver dyad severity scores were only moderately correlated (r = 0.54, adj. p < 0.001), with caregivers generally scoring higher than their children. Bullying and stigma were unaffected by age, gender, racial or ethnic background, school type, severity, or academic performance. Of all affected body parts, only facial involvement correlated significantly with increased child‐reported bullying (adj. p < 0.05; Table S1). Although ichthyosis subtype did not affect bullying, stigma significantly differed among the most represented subtypes (ANOVA, adj. p < 0.05); children with LI and IWC experienced more stigmatization than those with EI (p < 0.01 and 0.05, respectively, adjusted by Tukey test), despite no difference in mean self‐reported severity.

TABLE 2.

Bullying and psychosocial scores reported.

Measure	Reporter	Mean ± SD	% with at least moderately increased score a
Bullying (CABS‐9)	Child	14.9 ± 5.3	33%
	Proxy	17.5 ± 5.9	53% based on validated bands for child CABS‐9
FDLQI	Caregiver	9.5 ± 5.5	Severity bands not validated
Peer relationships	Child b	50.3 ± 7.7	8.3%
Depression	Child	46.5 ± 7.8	6.3%
	Caregiver	46.6 ± 8.1	6.3%
Fatigue	Child	44.8 ± 9.9	6.3%
Anxiety	Child	45.9 ± 8.3	6.3%
	Caregiver	49.9 ± 10.5	18.4%
Mobility	Child	47.2 ± 8.5	16.7%
Stigma	Child b	45.9 ± 8.2	60.4%

^a Child Adolescent Bullying Scale‐9 Question Version (CABS‐9) scores range from 9 to 36 [14]; for the Family Dermatology Life Quality Index (FDLQI), the range is 0–30, with higher scores representing greater impairment of QOL [17]. Patient‐Reported Outcomes Measurement Information System (PROMIS) scores ≥ 60 are increased for depression, fatigue, and anxiety; scores ≤ 40 are increased for mobility and peer relationships [15, 18]. Stigma scores are moderately high between 45 and 55 and high ≥ 55 [16].

^b One child did not answer enough questions in the PROMIS peer relationships and stigma sections to receive a score for these categories; these categories thus had only 48 responses.

3.2. Correlations of Bullying With Psychosocial Outcomes

Child‐reported bullying exposure correlated most strongly with poor peer relationships (r = −0.63) and stigma (r = 0.53) (both adj. p < 0.001). Stigma also correlated significantly with poor peer relationships and child depression (Figure 1). Caregiver (but not child) depression, both caregiver and child anxiety, and poor family QoL correlated with proxy‐reported bullying exposure (Figure 1). FDLQI scores correlated best with caregiver anxiety (r = 0.59, adj. p < 0.001) but also with proxy‐reported severity, poor child mobility, poor peer relationships, and caregiver and child depression. Child‐reported severity was not correlated with bullying and only weakly correlated with child depression, poor mobility, and FDLQI, suggesting the benefit of inquiring about bullying and its psychosocial impact, regardless of the extent of affected skin but certainly with facial involvement. Child and caregiver dyad bullying scores were only moderately correlated (r = 0.45, adj. p < 0.01), suggesting that children limit sharing bullying experiences and further supporting the need to capture input from both the child and caregiver.

FIGURE 1.

Heatmap of Spearman correlations comparing patient characteristics and patient/proxy severity, bullying, and psychosocial metrics. For most scores, more intense red means higher severity; exceptions are peer relationships and mobility, with more intense blue (and negative rho values) meaning poorer peer relationships and mobility. *p < 0.05; **p < 0.01; ***p < 0.001. Correlations of special interest are emphasized in bolding and boxes.

3.3. Patient Experiences With Bullying

Responses to the optional open‐ended question on bullying experiences were shared by 30 children and 35 caregivers. Bullying was perpetrated by other children in 80.7% of cases, by teachers in 7.7%, and by other adults in 11.5%. Nine themes emerged (Figure 2 and Table S2), largely reflecting negative reactions by others. Children were called names, asked rude questions, and excluded. Concerns about contagion led to mistaken diagnoses (leprosy, lice), fear of touching the child or holding hands, and even one situation in which security officers were called to escort the family out of a public space. Children received stares and negative attention and had their images posted online without permission. Children often fabricated excuses for their appearance, such as sunburn or scaling “from the pool.” Due to self‐consciousness about their skin, they wore long sleeves and pants during hot weather or hid their hands in pockets. Children reported feeling embarrassed, sad, worried about the future, and “less human.”

FIGURE 2.

Bullying themes identified from reported experiences of pediatric ichthyosis patients.

Caregivers suggested various ways to manage negative attention: (i) avoiding framing ichthyosis as a secret or shameful; (ii) explaining why people stare and highlighting the child's unique qualities; (iii) noting that others' questions may indicate something new, rather than something wrong; and (iv) sharing information when questioned only if comfortable. Others avoid appearance‐related comments at home and focus instead on character and action‐based comments.

4. Discussion

In this study, 33% of children with ichthyosis experienced moderate bullying, higher than the 15%–19% of the general US high school population per year between 2021 and 2023 [1]. Bullying exposure strongly correlated with poor peer relationships and moderately with stigma, which was experienced to a moderate extent in 60.4% and was severe in another 10.4%. Children with ichthyosis primarily reported verbal bullying. Even without malicious intent, comments about appearance were hurtful.

We hypothesized that high stigma and bullying would translate into poor mental health. Indeed, based on the strong Spearman correlations, those experiencing greater bullying had poorer peer relationships. However, mean psychosocial scores were within 0.5 SD of the mean, suggesting that children with ichthyosis since birth largely develop resilience and environmental support to cope with psychosocial challenges. Notably, 6.3% of children had at least moderately severe fatigue, depression, and anxiety, emphasizing the need for awareness and support.

Neither bullying nor stigma correlated with disease severity. Surprisingly, the strongest correlation with a psychosocial domain was the weak correlation of severity with depression (r = 0.36). These results suggest the greater importance of factors that contribute to bullying (and stigma) beyond disease severity and visibility. While the relatively small sample size and potential underpowering could have lowered correlations and the significance of results, our data mirror the weak correlations of severity with child‐reported stigma recently reported in a study with almost 1700 children with various chronic skin diseases [4].

Beyond the paucity of studies on bullying for ichthyosis and chronic skin diseases in general, little is known about the impact of a child's bullying on caregiver mental health. We found that proxies tended to overestimate the extent of bullying compared to the child and that caregiver depression, anxiety, and poor family QoL were moderately to strongly correlated with this higher proxy‐reported bullying score. These findings stress the importance of assessing the burden on the caregiver and family. As other studies have explored, dermatologists play a pivotal role in reducing this burden on families through approaches including trauma‐informed care and behavioral counseling [19].

Our data support incorporating bullying screening into visits to pediatricians and dermatologists for school‐aged children with ichthyosis [3, 20]. A standard bullying screening tool has not been established; however, providers can choose from available tools or ask relevant open‐ended questions [3, 19]. The American Academy of Pediatrics (AAP), American Academy of Family Physicians (AAFP), and American Academy of Child and Adolescent Psychiatry (AACAP) offer detailed resources on bullying management for families and healthcare providers [20, 21, 22]. In addition, camps and organizations for children with skin conditions provide enriching, supportive opportunities (Table S3). Ichthyosis‐specific and other children's books featuring characters with visible differences are also anti‐bullying tools for families and providers; these books are cataloged online through FIRST and Disability Book Week (Table S3). Increasing awareness regarding skin conditions like ichthyosis may also be approached through education in the school setting, by patients, family members, and specialists; indeed, some parents did share in our optional survey that this has been helpful as a proactive approach against bullying. At the legislative level, federal acts protect students with conditions such as ichthyosis from discrimination, including the Individual with Disabilities Education Act (IDEA) and Section 504 of the Education Act of 1973 [23, 24]. With awareness of this information, caregivers can better prepare affected children and their school environment, ensuring that there is a strong support network and proactively educating the child and school about ichthyosis and its lack of contagion.

5. Limitations

Our sample size was substantial for a rare disease, but still small and demographically homogeneous, which limits the generalizability of results. The COVID‐19 pandemic could have affected bullying reports and mental health in mid‐2022 [25]. The CABS‐9 questionnaire only captures recent bullying exposures, potentially missing historical or prolonged experiences. Furthermore, the CABS‐9 is not validated for children 8–10 years old or for caregivers/proxies, limiting the value of the data; however, there are no existing validated metrics for bullying for these populations.

6. Conclusions and Relevance

Bullying is frequently experienced by children with ichthyosis, regardless of disease severity. Bullying victimization negatively impacts the QoL and mental health of the child and caregivers. Pediatricians and dermatologists should consider assessing bullying and its consequences in children with ichthyosis during visits to provide holistic support.

Ethics Statement

Both Ann and the Robert H. Lurie Children's Hospital of Chicago and Northwestern University Institutional Review Boards approved the study.

Consent

Participating adults gave written, informed consent, and children 12 years of age or above gave written assent for this study.

Conflicts of Interest

Although not of relevance to this publication, A.S.P. has been an investigator without personal compensation for AbbVie, Applied Pharma Research, Dermavant, Eli Lilly, Incyte, Janssen, Krystal, Regeneron, Timber, and UCB; a consultant for AbbVie, Abeona, Apogee, Arcutis, Aslan, BioCryst, Boehringer‐Ingelheim, Bristol‐Myers‐Squibb, Dermavant, Incyte, Johnson and Johnson, Krystal Biotech, LEO, Mitsubishi Tanabe, Nektar, Primus, Procter and Gamble, Regeneron, Sanofi, Seanergy, TWI Biotech, and UCB; and on the data safety monitoring board for AbbVie, Abeona, and Galderma. The other authors declare no conflicts of interest.

Supporting information

Table S1.

Table S2.

Table S3.

Data Availability Statement

The data that support the findings of this study are available on request from the corresponding author. The data are not publicly available due to privacy or ethical restrictions.