Racial Disparities in Hidradenitis Suppurativa–Related Pain: A Cross-sectional Analysis

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Racial Disparities in Hidradenitis Suppurativa–Related Pain: A Cross-sectional Analysis

Hidradenitis suppurativa (HS), a chronic inflammatory disease that is characterized by tender inflamed nodules of the skin and subcutaneous tissue, disproportionately affects postpubertal females as well as Black/African American individuals. The nodules can rupture, form sinus tracts, and scar. 1 Hidradenitis suppurativa has been associated with cardiovascular disease, type 2 diabetes mellitus, polycystic ovary syndrome, depression, suicide, and substance use disorders. Because of the symptom burden and associated conditions, HS can be a painful and distressing disease that substantially impairs the quality of life for individuals with this condition. 2

Pain is a commonly reported symptom in HS that often goes untreated. Furthermore, HS-related pain is complex due to the involvement of different pain types that require various treatment modalities.3 According to Savage et al,4 recognizing whether HS-related pain is acute, chronic, neuropathic, or nociceptive is vital in establishing a framework for an effective pain management scheme. Currently, such established multimodal pain management strategies in dermatology do not exist. In 2021, dermatology-specific pain management strategies proposed the use of a multimodal regimen to address the multifaceted nature of HS-related pain.4 However, these strategies failed to recognize the systemic racial and ethnic biases in the US health care system that undermine pain management care for minority groups.5,6 One approach to combatting racial disparities in pain management is determining average pain levels across racial groups.7 This study sought to compare HS-related pain scores by racial groups. Furthermore, we assessed differences in perception of patients’ respective pain management regimens by race. We hypothesized that the average HS-related pain intensities and pain management would differ between self-reported racial groups.

Methods  

This cross-sectional study took place over 5 months (August through December 2021). A survey was emailed to 2198 adult patients with HS in the University of Alabama Health System. The survey consisted of demographic and general questions about a patient’s HS. Pain scores were captured using the numeric rating scale (NRS), a measurement tool for pain intensity on a scale from 0 to 10. 8 Age at diagnosis, gender, education level, household income, total body areas affected by HS, disease severity (categorized as mild, moderate, and severe), comorbidities including mood disorders, tobacco use, and HS and HS-related pain medication regimens also were collected. Additionally, participants were asked about their level of agreement with the following statements: “I am satisfied with how my pain related to HS is being managed by my doctors” and “My pain related to HS is under control.” The level of agreement was measured using a 5-point Likert scale, with responses ranging from strongly disagree to strongly agree. All data included in the analysis were self-reported. The study received institutional review board approval for the University of Alabama at Birmingham.

Statistical Analysis—Descriptive statistics were used to assess statistical differences in patient characteristics of Black/African American participants compared to other participants, including White, Asian, and Hispanic/Latino participants. Thirteen participants were excluded from the final analysis: 2 participants were missing data, and 11 biracial participants were excluded due to overlapping White and Black/African American races that may have confounded the analysis. Categorical variables were reported as frequencies and percentages, and χ2 and Fisher exact tests, when necessary, were used to test for statistically significant differences. Continuous variables were summarized with means and standard deviations, and a t test was used for statistically significant differences.

Logistic regression was performed to assess the relationship between race and pain after adjusting for confounding variables such as obesity, current tobacco use, self-reported HS severity, and the presence of comorbidities. A total of 204 patient records were included in the analysis, of which 70 (34.3%) had a pain score of 8 or higher, which indicates very severe pain intensity levels on the NRS,8 and were selected as a cut point based on the distribution of responses. For this cross-sectional cohort, our approach was to compare characteristics of those classified with a top score of 8 or higher (n=70) vs a top score of 0 to 7 (n=134)(cases vs noncases). Statistical analyses were performed using JMP Pro 16 (JMP Statistical Discovery LLC) at an α=.05 significance level; logistic regression was performed using SPSS Statistics (IBM). For the logistic regression, we grouped patient race into 2 categories: Black/African American and Other, which included White, Asian, and Hispanic/Latino participants.

Crude and adjusted multivariable logistic regression analyses were used to calculate prevalence odds ratios with 95% confidence intervals. Covariate inclusion in the multivariable logistic regression was based on a priori hypothesis/knowledge and was meant to estimate the independent effect of race after adjustment for income, HS severity, and history of prescription pain medication use. Other variables, including tobacco use, obesity, mood disorders, and current HS treatments, were all individually tested in the multivariate analysis and did not significantly impact the odds ratio for high pain. Statistical adjustment slightly decreased (19%) the magnitude between crude and adjusted prevalence odds ratios for the association between Black/African American race and high pain score.

Results  

Survey Demographics —The final analysis included 204 survey respondents. Most respondents were Black/African American (58.82%), and nearly all were female (89.71%)(Table 1). The mean age (SD) of respondents was 37.37 (11.29) years (range, 19-70 years). Many respondents reported having completed some college (36.27%) or receiving a bachelor’s degree (19.12%). Of patients who were not Black/African American, 10.71% had higher than a master’s degree, whereas no Black/African American patients held a degree higher than a master’s ( P = .0052). Additionally, more Black/African American respondents (35.83%) reported an annual household income level of less than $25,000 compared with respondents who were not Black/African American (19.05%, P = .0001). Most respondents rated the severity of their HS as moderate or severe (46.57% and 41.67%, respectively), and there was no significant difference in reported severity of HS between racial groups ( P = .5395).

Study Sample Characteristics by Race

Study Sample Characteristics by Race

 

 

Pain Scores—As documented in the Methods, respondents were asked to rate their HS-related pain intensity from 0 to 10 using the NRS. The average pain score (SD)—the level of pain intensity over the prior month—was 6.39 (2.56)(range, 0–10). The mean pain score (SD) at the time of the survey was 3.61 (2.98)(range, 0–10)(Table 1). These data revealed that Black/African American patients had a significantly higher average pain score (SD) than patients with HS who were not Black/African American (7.08 [2.49] and 5.40 [2.35], respectively; P<.0001). After adjustment with multivariable logistical regression, Black/African American patients had 4-fold increased odds for very severe levels of pain (score of ≥8) compared with patients who were not Black/African American.

Pain ManagementAlthough pain scores were higher for Black/African American patients with HS, there was no significant difference in the perception of pain control between racial groups (P=.0761). Additionally, we found low income (adjusted prevalence odds ratio [POR], 0.22; 95% CI, 0.05-0.91), a history of prescription pain medication use (adjusted POR, 2.25; 95% CI, 1.13-4.51), and HS severity (adjusted POR, 4.40; 95% CI, 1.11-17.36) all to be independent risk factors contributing to higher pain scores in patients with HS (Table 2). Lastly, we noted current or reported history of pain medication use was significantly correlated with higher pain scores (P=.0280 and P=.0213, respectively).

Results From Multivariable Logistic Regression for the Association Between Select Patient Characteristics and High Pain Score (N=204)

Satisfaction With Pain ManagementThe level of satisfaction with physician management of HS-related pain was significantly different between Black/African American patients and those who were not Black/African American (P=.0129). Of those who identified as Black/African American, 26.7% (n=32) strongly disagreed with the statement, “I am satisfied with how my pain related to HS is being managed by my doctors,” whereas only 15.5% (n=13) of patients who were not Black/African American strongly disagreed. 

Comment

There is no cure for HS, and a large focus of treatment is pain management. Because racial disparities in the treatment of chronic pain will affect those with HS, we conducted a cross-sectional analysis of pain and pain management among HS patients. We found that Black/African American patients with HS have higher average pain scores than those who are not Black/African American and were 4 times more likely to experience very severe pain. Prior studies have established that patients with HS often report higher pain levels than patients with other chronic inflammatory skin conditions, 7,8 and our study identified racial disparities in HS-related pain management.

Measuring pain is challenging because of its multidimensional and subjective nature, making it essential to consider underlying causes and patients’ emotional responses to pain.9 By adjusting for confounding factors that may influence pain, such as mood disorders, disease severity, comorbidities, and medication use, we were able to gain better insight into fundamental differences in average pain intensity levels among racial groups and assess what factors may be contributing to a patient’s pain perception. Our study determined that lower income levels, higher HS disease severity, and a history of prescription pain medication use were all independent risk factors for high pain. Of note, obesity, tobacco use, and mood disorders such as anxiety and depression did not significantly differ between racial groups or increase the odds of high pain between racial groups identified.

With low income being an independent risk factor for high pain, we must consider the social determinants of health and how they may influence the pain experience in HS. We speculate that low income may be associated with other social determinants of health for the patients assessed in this study, such as lack of social and community support or limited health care access that contribute to worse health outcomes.10,11 In addition, low income contributes to limited access to medical care or treatments12; without access to effective HS management, lower-income patients may be at risk for higher disease severity and thus higher pain levels. However, economic stability is only a part of the whole picture; therefore, assessing the other social determinants of health in patients with HS may lead to better health outcomes and quality of life.

Another identified risk factor for high pain was a reported history of prescription pain medication use. This finding suggests that patients with moderate to severe pain likely have required stronger analgesic medications in the past. We further speculate that high pain levels in patients who have received prescription pain medications indicate either undertreatment, mistreatment, or recalcitrant pain. More research is needed to assess the relationship between HS-related pain intensity, analgesic medications, and providers who manage HS-related pain.

We also found that Black/African American patients with HS had a significantly higher dissatisfaction with their physician’s management of their pain, which could be attributable to several factors, including biological differences in medication metabolism (in which the patient has medication-resistant HS), undertreatment of pain, and/or poor doctor-patient relations. These reasons coincide with other diseases where health disparities are found.13-15 Recognizing these factors will be key to dismantling the disparities in HS that are noted within this study. The limitations of this work include the cross-sectional study design and its inability to evaluate causal factors of high pain levels across racial groups, the NRS lack of insight on pain chronicity or pain experience,7 the lack of provider or institution perspectives, and self-reported data. Additionally, only patients with email access were included, which may have excluded vulnerable populations with more pain associated with their HS.

Our findings highlight an area for further investigation to assess why these racial differences exist in HS-related pain. The results also emphasize the need for research evaluating whether systemic or health care provider biases contribute to racial differences in HS-related pain management.

Acknowledgment Dr. Weir was supported by the Predoctoral Clinical/Translational Research Program (TL1), a National Institutes of Health Ruth L. Kirschstein National Research Service Award (NRSA), through the University of Alabama at Birmingham (UAB) Center for Clinical and Translational Science (CCTS).  

References
  1. Garg A, Kirby JS, Lavian J, et al. Sex- and age-adjusted population analysis of prevalence estimates for hidradenitis suppurativa in the United States. JAMA Dermatol. 2017;153:760-764. doi:10.1001/jamadermatol.2017.0201
  2. Nguyen TV, Damiani G, Orenstein LAV, et al. Hidradenitis suppurativa: an update on epidemiology, phenotypes, diagnosis, pathogenesis, comorbidities and quality of life. J Eur Acad Dermatol Venereol. 2021;35:50-61. doi:10.1111/jdv.16677
  3. Krajewski PK, Matusiak Ł, von Stebut E, et al. Pain in hidradenitis suppurativa: a cross-sectional study of 1,795 patients. Acta Derm Venereol. 2021;101:adv00364. doi:10.2340/00015555-3724
  4. Savage KT, Singh V, Patel ZS, et al. Pain management in hidradenitis suppurativa and a proposed treatment algorithm. J Am Acad Dermatol. 2021;85:187-199. doi:10.1016/j.jaad.2020.09.039
  5. Morales ME, Yong RJ. Racial and ethnic disparities in the treatment of chronic pain. Pain Med. 2021;22:75-90. doi:10.1093/pm/pnaa427
  6. US Department of Health and Human Services. 2019 National Healthcare Quality and Disparities Report. December 2020. Accessed June 21, 2023. https://www.ahrq.gov/sites/default/files/wysiwyg/research/findings/nhqrdr/2019qdr.pdf
  7. Hoffman KM, Trawalter S, Axt JR, et al. Racial bias in pain assessment and treatment recommendations, and false beliefs about biological differences between blacks and whites. Proc Natl Acad Sci U S A. 2016;113:4296-4301. doi:10.1073/pnas.1516047113
  8. Patel ZS, Hoffman LK, Buse DC, et al. Pain, psychological comorbidities, disability, and impaired quality of life in hidradenitis suppurativa. Curr Pain Headache Rep. 2017;21:49. doi:10.1007/s11916-017-0647-3. Published correction appears in Curr Pain Headache Rep. 2017;21:52.
  9. McDowell I. Pain measurements. In: Measuring Health: A Guide to Rating Scales and Questionnaires. Oxford University Press; 2006:477-478.
  10. Singh GK, Daus GP, Allender M, et al. Social determinants of health in the United States: addressing major health inequality trends for the nation, 1935-2016. Int J MCH AIDS. 2017;6:139-164. doi:10.21106/ijma.236
  11. Sulley S, Bayssie M. Social determinants of health: an evaluation of risk factors associated with inpatient presentations in the United States. Cureus. 2021;13:E13287. doi:10.7759/cureus.13287
  12. Lazar M, Davenport L. Barriers to health care access for low income families: a review of literature. J Community Health Nurs. 2018;35:28-37. doi:10.1080/07370016.2018.1404832
  13. Ghoshal M, Shapiro H, Todd K, et al. Chronic noncancer pain management and systemic racism: time to move toward equal care standards.J Pain Res. 2020;13:2825-2836. doi:10.214/JPR.S287314
  14. Cintron A, Morrison RS. Pain and ethnicity in the United States: a systematic review. J Palliat Med. 2006;9:1454-1473. doi:10.1089/jpm.2006.9.1454
  15. Green CR, Anderson KO, Baker TA, et al. The unequal burden of pain: confronting racial and ethnic disparities in pain. Pain Med. 2003;4:277-294. doi:10.1046/j.1526-4637.2003.03034.x. Published correction appears in Pain Med. 2005;6:99.
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From the University of Alabama at Birmingham. Dr. Weir is from the Marnix E. Heersink School of Medicine; Dr. MacLennan is from the Department of Surgery, Division of Transplantation; and Dr. Kole is from the Department of Dermatology.

The authors report no conflict of interest.

Correspondence: Sydney Alexis Weir, MD, MSPH, 500 22nd St S, Floor 3, Birmingham, AL 35233 ([email protected]).

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From the University of Alabama at Birmingham. Dr. Weir is from the Marnix E. Heersink School of Medicine; Dr. MacLennan is from the Department of Surgery, Division of Transplantation; and Dr. Kole is from the Department of Dermatology.

The authors report no conflict of interest.

Correspondence: Sydney Alexis Weir, MD, MSPH, 500 22nd St S, Floor 3, Birmingham, AL 35233 ([email protected]).

Author and Disclosure Information

From the University of Alabama at Birmingham. Dr. Weir is from the Marnix E. Heersink School of Medicine; Dr. MacLennan is from the Department of Surgery, Division of Transplantation; and Dr. Kole is from the Department of Dermatology.

The authors report no conflict of interest.

Correspondence: Sydney Alexis Weir, MD, MSPH, 500 22nd St S, Floor 3, Birmingham, AL 35233 ([email protected]).

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Hidradenitis suppurativa (HS), a chronic inflammatory disease that is characterized by tender inflamed nodules of the skin and subcutaneous tissue, disproportionately affects postpubertal females as well as Black/African American individuals. The nodules can rupture, form sinus tracts, and scar. 1 Hidradenitis suppurativa has been associated with cardiovascular disease, type 2 diabetes mellitus, polycystic ovary syndrome, depression, suicide, and substance use disorders. Because of the symptom burden and associated conditions, HS can be a painful and distressing disease that substantially impairs the quality of life for individuals with this condition. 2

Pain is a commonly reported symptom in HS that often goes untreated. Furthermore, HS-related pain is complex due to the involvement of different pain types that require various treatment modalities.3 According to Savage et al,4 recognizing whether HS-related pain is acute, chronic, neuropathic, or nociceptive is vital in establishing a framework for an effective pain management scheme. Currently, such established multimodal pain management strategies in dermatology do not exist. In 2021, dermatology-specific pain management strategies proposed the use of a multimodal regimen to address the multifaceted nature of HS-related pain.4 However, these strategies failed to recognize the systemic racial and ethnic biases in the US health care system that undermine pain management care for minority groups.5,6 One approach to combatting racial disparities in pain management is determining average pain levels across racial groups.7 This study sought to compare HS-related pain scores by racial groups. Furthermore, we assessed differences in perception of patients’ respective pain management regimens by race. We hypothesized that the average HS-related pain intensities and pain management would differ between self-reported racial groups.

Methods  

This cross-sectional study took place over 5 months (August through December 2021). A survey was emailed to 2198 adult patients with HS in the University of Alabama Health System. The survey consisted of demographic and general questions about a patient’s HS. Pain scores were captured using the numeric rating scale (NRS), a measurement tool for pain intensity on a scale from 0 to 10. 8 Age at diagnosis, gender, education level, household income, total body areas affected by HS, disease severity (categorized as mild, moderate, and severe), comorbidities including mood disorders, tobacco use, and HS and HS-related pain medication regimens also were collected. Additionally, participants were asked about their level of agreement with the following statements: “I am satisfied with how my pain related to HS is being managed by my doctors” and “My pain related to HS is under control.” The level of agreement was measured using a 5-point Likert scale, with responses ranging from strongly disagree to strongly agree. All data included in the analysis were self-reported. The study received institutional review board approval for the University of Alabama at Birmingham.

Statistical Analysis—Descriptive statistics were used to assess statistical differences in patient characteristics of Black/African American participants compared to other participants, including White, Asian, and Hispanic/Latino participants. Thirteen participants were excluded from the final analysis: 2 participants were missing data, and 11 biracial participants were excluded due to overlapping White and Black/African American races that may have confounded the analysis. Categorical variables were reported as frequencies and percentages, and χ2 and Fisher exact tests, when necessary, were used to test for statistically significant differences. Continuous variables were summarized with means and standard deviations, and a t test was used for statistically significant differences.

Logistic regression was performed to assess the relationship between race and pain after adjusting for confounding variables such as obesity, current tobacco use, self-reported HS severity, and the presence of comorbidities. A total of 204 patient records were included in the analysis, of which 70 (34.3%) had a pain score of 8 or higher, which indicates very severe pain intensity levels on the NRS,8 and were selected as a cut point based on the distribution of responses. For this cross-sectional cohort, our approach was to compare characteristics of those classified with a top score of 8 or higher (n=70) vs a top score of 0 to 7 (n=134)(cases vs noncases). Statistical analyses were performed using JMP Pro 16 (JMP Statistical Discovery LLC) at an α=.05 significance level; logistic regression was performed using SPSS Statistics (IBM). For the logistic regression, we grouped patient race into 2 categories: Black/African American and Other, which included White, Asian, and Hispanic/Latino participants.

Crude and adjusted multivariable logistic regression analyses were used to calculate prevalence odds ratios with 95% confidence intervals. Covariate inclusion in the multivariable logistic regression was based on a priori hypothesis/knowledge and was meant to estimate the independent effect of race after adjustment for income, HS severity, and history of prescription pain medication use. Other variables, including tobacco use, obesity, mood disorders, and current HS treatments, were all individually tested in the multivariate analysis and did not significantly impact the odds ratio for high pain. Statistical adjustment slightly decreased (19%) the magnitude between crude and adjusted prevalence odds ratios for the association between Black/African American race and high pain score.

Results  

Survey Demographics —The final analysis included 204 survey respondents. Most respondents were Black/African American (58.82%), and nearly all were female (89.71%)(Table 1). The mean age (SD) of respondents was 37.37 (11.29) years (range, 19-70 years). Many respondents reported having completed some college (36.27%) or receiving a bachelor’s degree (19.12%). Of patients who were not Black/African American, 10.71% had higher than a master’s degree, whereas no Black/African American patients held a degree higher than a master’s ( P = .0052). Additionally, more Black/African American respondents (35.83%) reported an annual household income level of less than $25,000 compared with respondents who were not Black/African American (19.05%, P = .0001). Most respondents rated the severity of their HS as moderate or severe (46.57% and 41.67%, respectively), and there was no significant difference in reported severity of HS between racial groups ( P = .5395).

Study Sample Characteristics by Race

Study Sample Characteristics by Race

 

 

Pain Scores—As documented in the Methods, respondents were asked to rate their HS-related pain intensity from 0 to 10 using the NRS. The average pain score (SD)—the level of pain intensity over the prior month—was 6.39 (2.56)(range, 0–10). The mean pain score (SD) at the time of the survey was 3.61 (2.98)(range, 0–10)(Table 1). These data revealed that Black/African American patients had a significantly higher average pain score (SD) than patients with HS who were not Black/African American (7.08 [2.49] and 5.40 [2.35], respectively; P<.0001). After adjustment with multivariable logistical regression, Black/African American patients had 4-fold increased odds for very severe levels of pain (score of ≥8) compared with patients who were not Black/African American.

Pain ManagementAlthough pain scores were higher for Black/African American patients with HS, there was no significant difference in the perception of pain control between racial groups (P=.0761). Additionally, we found low income (adjusted prevalence odds ratio [POR], 0.22; 95% CI, 0.05-0.91), a history of prescription pain medication use (adjusted POR, 2.25; 95% CI, 1.13-4.51), and HS severity (adjusted POR, 4.40; 95% CI, 1.11-17.36) all to be independent risk factors contributing to higher pain scores in patients with HS (Table 2). Lastly, we noted current or reported history of pain medication use was significantly correlated with higher pain scores (P=.0280 and P=.0213, respectively).

Results From Multivariable Logistic Regression for the Association Between Select Patient Characteristics and High Pain Score (N=204)

Satisfaction With Pain ManagementThe level of satisfaction with physician management of HS-related pain was significantly different between Black/African American patients and those who were not Black/African American (P=.0129). Of those who identified as Black/African American, 26.7% (n=32) strongly disagreed with the statement, “I am satisfied with how my pain related to HS is being managed by my doctors,” whereas only 15.5% (n=13) of patients who were not Black/African American strongly disagreed. 

Comment

There is no cure for HS, and a large focus of treatment is pain management. Because racial disparities in the treatment of chronic pain will affect those with HS, we conducted a cross-sectional analysis of pain and pain management among HS patients. We found that Black/African American patients with HS have higher average pain scores than those who are not Black/African American and were 4 times more likely to experience very severe pain. Prior studies have established that patients with HS often report higher pain levels than patients with other chronic inflammatory skin conditions, 7,8 and our study identified racial disparities in HS-related pain management.

Measuring pain is challenging because of its multidimensional and subjective nature, making it essential to consider underlying causes and patients’ emotional responses to pain.9 By adjusting for confounding factors that may influence pain, such as mood disorders, disease severity, comorbidities, and medication use, we were able to gain better insight into fundamental differences in average pain intensity levels among racial groups and assess what factors may be contributing to a patient’s pain perception. Our study determined that lower income levels, higher HS disease severity, and a history of prescription pain medication use were all independent risk factors for high pain. Of note, obesity, tobacco use, and mood disorders such as anxiety and depression did not significantly differ between racial groups or increase the odds of high pain between racial groups identified.

With low income being an independent risk factor for high pain, we must consider the social determinants of health and how they may influence the pain experience in HS. We speculate that low income may be associated with other social determinants of health for the patients assessed in this study, such as lack of social and community support or limited health care access that contribute to worse health outcomes.10,11 In addition, low income contributes to limited access to medical care or treatments12; without access to effective HS management, lower-income patients may be at risk for higher disease severity and thus higher pain levels. However, economic stability is only a part of the whole picture; therefore, assessing the other social determinants of health in patients with HS may lead to better health outcomes and quality of life.

Another identified risk factor for high pain was a reported history of prescription pain medication use. This finding suggests that patients with moderate to severe pain likely have required stronger analgesic medications in the past. We further speculate that high pain levels in patients who have received prescription pain medications indicate either undertreatment, mistreatment, or recalcitrant pain. More research is needed to assess the relationship between HS-related pain intensity, analgesic medications, and providers who manage HS-related pain.

We also found that Black/African American patients with HS had a significantly higher dissatisfaction with their physician’s management of their pain, which could be attributable to several factors, including biological differences in medication metabolism (in which the patient has medication-resistant HS), undertreatment of pain, and/or poor doctor-patient relations. These reasons coincide with other diseases where health disparities are found.13-15 Recognizing these factors will be key to dismantling the disparities in HS that are noted within this study. The limitations of this work include the cross-sectional study design and its inability to evaluate causal factors of high pain levels across racial groups, the NRS lack of insight on pain chronicity or pain experience,7 the lack of provider or institution perspectives, and self-reported data. Additionally, only patients with email access were included, which may have excluded vulnerable populations with more pain associated with their HS.

Our findings highlight an area for further investigation to assess why these racial differences exist in HS-related pain. The results also emphasize the need for research evaluating whether systemic or health care provider biases contribute to racial differences in HS-related pain management.

Acknowledgment Dr. Weir was supported by the Predoctoral Clinical/Translational Research Program (TL1), a National Institutes of Health Ruth L. Kirschstein National Research Service Award (NRSA), through the University of Alabama at Birmingham (UAB) Center for Clinical and Translational Science (CCTS).  

Hidradenitis suppurativa (HS), a chronic inflammatory disease that is characterized by tender inflamed nodules of the skin and subcutaneous tissue, disproportionately affects postpubertal females as well as Black/African American individuals. The nodules can rupture, form sinus tracts, and scar. 1 Hidradenitis suppurativa has been associated with cardiovascular disease, type 2 diabetes mellitus, polycystic ovary syndrome, depression, suicide, and substance use disorders. Because of the symptom burden and associated conditions, HS can be a painful and distressing disease that substantially impairs the quality of life for individuals with this condition. 2

Pain is a commonly reported symptom in HS that often goes untreated. Furthermore, HS-related pain is complex due to the involvement of different pain types that require various treatment modalities.3 According to Savage et al,4 recognizing whether HS-related pain is acute, chronic, neuropathic, or nociceptive is vital in establishing a framework for an effective pain management scheme. Currently, such established multimodal pain management strategies in dermatology do not exist. In 2021, dermatology-specific pain management strategies proposed the use of a multimodal regimen to address the multifaceted nature of HS-related pain.4 However, these strategies failed to recognize the systemic racial and ethnic biases in the US health care system that undermine pain management care for minority groups.5,6 One approach to combatting racial disparities in pain management is determining average pain levels across racial groups.7 This study sought to compare HS-related pain scores by racial groups. Furthermore, we assessed differences in perception of patients’ respective pain management regimens by race. We hypothesized that the average HS-related pain intensities and pain management would differ between self-reported racial groups.

Methods  

This cross-sectional study took place over 5 months (August through December 2021). A survey was emailed to 2198 adult patients with HS in the University of Alabama Health System. The survey consisted of demographic and general questions about a patient’s HS. Pain scores were captured using the numeric rating scale (NRS), a measurement tool for pain intensity on a scale from 0 to 10. 8 Age at diagnosis, gender, education level, household income, total body areas affected by HS, disease severity (categorized as mild, moderate, and severe), comorbidities including mood disorders, tobacco use, and HS and HS-related pain medication regimens also were collected. Additionally, participants were asked about their level of agreement with the following statements: “I am satisfied with how my pain related to HS is being managed by my doctors” and “My pain related to HS is under control.” The level of agreement was measured using a 5-point Likert scale, with responses ranging from strongly disagree to strongly agree. All data included in the analysis were self-reported. The study received institutional review board approval for the University of Alabama at Birmingham.

Statistical Analysis—Descriptive statistics were used to assess statistical differences in patient characteristics of Black/African American participants compared to other participants, including White, Asian, and Hispanic/Latino participants. Thirteen participants were excluded from the final analysis: 2 participants were missing data, and 11 biracial participants were excluded due to overlapping White and Black/African American races that may have confounded the analysis. Categorical variables were reported as frequencies and percentages, and χ2 and Fisher exact tests, when necessary, were used to test for statistically significant differences. Continuous variables were summarized with means and standard deviations, and a t test was used for statistically significant differences.

Logistic regression was performed to assess the relationship between race and pain after adjusting for confounding variables such as obesity, current tobacco use, self-reported HS severity, and the presence of comorbidities. A total of 204 patient records were included in the analysis, of which 70 (34.3%) had a pain score of 8 or higher, which indicates very severe pain intensity levels on the NRS,8 and were selected as a cut point based on the distribution of responses. For this cross-sectional cohort, our approach was to compare characteristics of those classified with a top score of 8 or higher (n=70) vs a top score of 0 to 7 (n=134)(cases vs noncases). Statistical analyses were performed using JMP Pro 16 (JMP Statistical Discovery LLC) at an α=.05 significance level; logistic regression was performed using SPSS Statistics (IBM). For the logistic regression, we grouped patient race into 2 categories: Black/African American and Other, which included White, Asian, and Hispanic/Latino participants.

Crude and adjusted multivariable logistic regression analyses were used to calculate prevalence odds ratios with 95% confidence intervals. Covariate inclusion in the multivariable logistic regression was based on a priori hypothesis/knowledge and was meant to estimate the independent effect of race after adjustment for income, HS severity, and history of prescription pain medication use. Other variables, including tobacco use, obesity, mood disorders, and current HS treatments, were all individually tested in the multivariate analysis and did not significantly impact the odds ratio for high pain. Statistical adjustment slightly decreased (19%) the magnitude between crude and adjusted prevalence odds ratios for the association between Black/African American race and high pain score.

Results  

Survey Demographics —The final analysis included 204 survey respondents. Most respondents were Black/African American (58.82%), and nearly all were female (89.71%)(Table 1). The mean age (SD) of respondents was 37.37 (11.29) years (range, 19-70 years). Many respondents reported having completed some college (36.27%) or receiving a bachelor’s degree (19.12%). Of patients who were not Black/African American, 10.71% had higher than a master’s degree, whereas no Black/African American patients held a degree higher than a master’s ( P = .0052). Additionally, more Black/African American respondents (35.83%) reported an annual household income level of less than $25,000 compared with respondents who were not Black/African American (19.05%, P = .0001). Most respondents rated the severity of their HS as moderate or severe (46.57% and 41.67%, respectively), and there was no significant difference in reported severity of HS between racial groups ( P = .5395).

Study Sample Characteristics by Race

Study Sample Characteristics by Race

 

 

Pain Scores—As documented in the Methods, respondents were asked to rate their HS-related pain intensity from 0 to 10 using the NRS. The average pain score (SD)—the level of pain intensity over the prior month—was 6.39 (2.56)(range, 0–10). The mean pain score (SD) at the time of the survey was 3.61 (2.98)(range, 0–10)(Table 1). These data revealed that Black/African American patients had a significantly higher average pain score (SD) than patients with HS who were not Black/African American (7.08 [2.49] and 5.40 [2.35], respectively; P<.0001). After adjustment with multivariable logistical regression, Black/African American patients had 4-fold increased odds for very severe levels of pain (score of ≥8) compared with patients who were not Black/African American.

Pain ManagementAlthough pain scores were higher for Black/African American patients with HS, there was no significant difference in the perception of pain control between racial groups (P=.0761). Additionally, we found low income (adjusted prevalence odds ratio [POR], 0.22; 95% CI, 0.05-0.91), a history of prescription pain medication use (adjusted POR, 2.25; 95% CI, 1.13-4.51), and HS severity (adjusted POR, 4.40; 95% CI, 1.11-17.36) all to be independent risk factors contributing to higher pain scores in patients with HS (Table 2). Lastly, we noted current or reported history of pain medication use was significantly correlated with higher pain scores (P=.0280 and P=.0213, respectively).

Results From Multivariable Logistic Regression for the Association Between Select Patient Characteristics and High Pain Score (N=204)

Satisfaction With Pain ManagementThe level of satisfaction with physician management of HS-related pain was significantly different between Black/African American patients and those who were not Black/African American (P=.0129). Of those who identified as Black/African American, 26.7% (n=32) strongly disagreed with the statement, “I am satisfied with how my pain related to HS is being managed by my doctors,” whereas only 15.5% (n=13) of patients who were not Black/African American strongly disagreed. 

Comment

There is no cure for HS, and a large focus of treatment is pain management. Because racial disparities in the treatment of chronic pain will affect those with HS, we conducted a cross-sectional analysis of pain and pain management among HS patients. We found that Black/African American patients with HS have higher average pain scores than those who are not Black/African American and were 4 times more likely to experience very severe pain. Prior studies have established that patients with HS often report higher pain levels than patients with other chronic inflammatory skin conditions, 7,8 and our study identified racial disparities in HS-related pain management.

Measuring pain is challenging because of its multidimensional and subjective nature, making it essential to consider underlying causes and patients’ emotional responses to pain.9 By adjusting for confounding factors that may influence pain, such as mood disorders, disease severity, comorbidities, and medication use, we were able to gain better insight into fundamental differences in average pain intensity levels among racial groups and assess what factors may be contributing to a patient’s pain perception. Our study determined that lower income levels, higher HS disease severity, and a history of prescription pain medication use were all independent risk factors for high pain. Of note, obesity, tobacco use, and mood disorders such as anxiety and depression did not significantly differ between racial groups or increase the odds of high pain between racial groups identified.

With low income being an independent risk factor for high pain, we must consider the social determinants of health and how they may influence the pain experience in HS. We speculate that low income may be associated with other social determinants of health for the patients assessed in this study, such as lack of social and community support or limited health care access that contribute to worse health outcomes.10,11 In addition, low income contributes to limited access to medical care or treatments12; without access to effective HS management, lower-income patients may be at risk for higher disease severity and thus higher pain levels. However, economic stability is only a part of the whole picture; therefore, assessing the other social determinants of health in patients with HS may lead to better health outcomes and quality of life.

Another identified risk factor for high pain was a reported history of prescription pain medication use. This finding suggests that patients with moderate to severe pain likely have required stronger analgesic medications in the past. We further speculate that high pain levels in patients who have received prescription pain medications indicate either undertreatment, mistreatment, or recalcitrant pain. More research is needed to assess the relationship between HS-related pain intensity, analgesic medications, and providers who manage HS-related pain.

We also found that Black/African American patients with HS had a significantly higher dissatisfaction with their physician’s management of their pain, which could be attributable to several factors, including biological differences in medication metabolism (in which the patient has medication-resistant HS), undertreatment of pain, and/or poor doctor-patient relations. These reasons coincide with other diseases where health disparities are found.13-15 Recognizing these factors will be key to dismantling the disparities in HS that are noted within this study. The limitations of this work include the cross-sectional study design and its inability to evaluate causal factors of high pain levels across racial groups, the NRS lack of insight on pain chronicity or pain experience,7 the lack of provider or institution perspectives, and self-reported data. Additionally, only patients with email access were included, which may have excluded vulnerable populations with more pain associated with their HS.

Our findings highlight an area for further investigation to assess why these racial differences exist in HS-related pain. The results also emphasize the need for research evaluating whether systemic or health care provider biases contribute to racial differences in HS-related pain management.

Acknowledgment Dr. Weir was supported by the Predoctoral Clinical/Translational Research Program (TL1), a National Institutes of Health Ruth L. Kirschstein National Research Service Award (NRSA), through the University of Alabama at Birmingham (UAB) Center for Clinical and Translational Science (CCTS).  

References
  1. Garg A, Kirby JS, Lavian J, et al. Sex- and age-adjusted population analysis of prevalence estimates for hidradenitis suppurativa in the United States. JAMA Dermatol. 2017;153:760-764. doi:10.1001/jamadermatol.2017.0201
  2. Nguyen TV, Damiani G, Orenstein LAV, et al. Hidradenitis suppurativa: an update on epidemiology, phenotypes, diagnosis, pathogenesis, comorbidities and quality of life. J Eur Acad Dermatol Venereol. 2021;35:50-61. doi:10.1111/jdv.16677
  3. Krajewski PK, Matusiak Ł, von Stebut E, et al. Pain in hidradenitis suppurativa: a cross-sectional study of 1,795 patients. Acta Derm Venereol. 2021;101:adv00364. doi:10.2340/00015555-3724
  4. Savage KT, Singh V, Patel ZS, et al. Pain management in hidradenitis suppurativa and a proposed treatment algorithm. J Am Acad Dermatol. 2021;85:187-199. doi:10.1016/j.jaad.2020.09.039
  5. Morales ME, Yong RJ. Racial and ethnic disparities in the treatment of chronic pain. Pain Med. 2021;22:75-90. doi:10.1093/pm/pnaa427
  6. US Department of Health and Human Services. 2019 National Healthcare Quality and Disparities Report. December 2020. Accessed June 21, 2023. https://www.ahrq.gov/sites/default/files/wysiwyg/research/findings/nhqrdr/2019qdr.pdf
  7. Hoffman KM, Trawalter S, Axt JR, et al. Racial bias in pain assessment and treatment recommendations, and false beliefs about biological differences between blacks and whites. Proc Natl Acad Sci U S A. 2016;113:4296-4301. doi:10.1073/pnas.1516047113
  8. Patel ZS, Hoffman LK, Buse DC, et al. Pain, psychological comorbidities, disability, and impaired quality of life in hidradenitis suppurativa. Curr Pain Headache Rep. 2017;21:49. doi:10.1007/s11916-017-0647-3. Published correction appears in Curr Pain Headache Rep. 2017;21:52.
  9. McDowell I. Pain measurements. In: Measuring Health: A Guide to Rating Scales and Questionnaires. Oxford University Press; 2006:477-478.
  10. Singh GK, Daus GP, Allender M, et al. Social determinants of health in the United States: addressing major health inequality trends for the nation, 1935-2016. Int J MCH AIDS. 2017;6:139-164. doi:10.21106/ijma.236
  11. Sulley S, Bayssie M. Social determinants of health: an evaluation of risk factors associated with inpatient presentations in the United States. Cureus. 2021;13:E13287. doi:10.7759/cureus.13287
  12. Lazar M, Davenport L. Barriers to health care access for low income families: a review of literature. J Community Health Nurs. 2018;35:28-37. doi:10.1080/07370016.2018.1404832
  13. Ghoshal M, Shapiro H, Todd K, et al. Chronic noncancer pain management and systemic racism: time to move toward equal care standards.J Pain Res. 2020;13:2825-2836. doi:10.214/JPR.S287314
  14. Cintron A, Morrison RS. Pain and ethnicity in the United States: a systematic review. J Palliat Med. 2006;9:1454-1473. doi:10.1089/jpm.2006.9.1454
  15. Green CR, Anderson KO, Baker TA, et al. The unequal burden of pain: confronting racial and ethnic disparities in pain. Pain Med. 2003;4:277-294. doi:10.1046/j.1526-4637.2003.03034.x. Published correction appears in Pain Med. 2005;6:99.
References
  1. Garg A, Kirby JS, Lavian J, et al. Sex- and age-adjusted population analysis of prevalence estimates for hidradenitis suppurativa in the United States. JAMA Dermatol. 2017;153:760-764. doi:10.1001/jamadermatol.2017.0201
  2. Nguyen TV, Damiani G, Orenstein LAV, et al. Hidradenitis suppurativa: an update on epidemiology, phenotypes, diagnosis, pathogenesis, comorbidities and quality of life. J Eur Acad Dermatol Venereol. 2021;35:50-61. doi:10.1111/jdv.16677
  3. Krajewski PK, Matusiak Ł, von Stebut E, et al. Pain in hidradenitis suppurativa: a cross-sectional study of 1,795 patients. Acta Derm Venereol. 2021;101:adv00364. doi:10.2340/00015555-3724
  4. Savage KT, Singh V, Patel ZS, et al. Pain management in hidradenitis suppurativa and a proposed treatment algorithm. J Am Acad Dermatol. 2021;85:187-199. doi:10.1016/j.jaad.2020.09.039
  5. Morales ME, Yong RJ. Racial and ethnic disparities in the treatment of chronic pain. Pain Med. 2021;22:75-90. doi:10.1093/pm/pnaa427
  6. US Department of Health and Human Services. 2019 National Healthcare Quality and Disparities Report. December 2020. Accessed June 21, 2023. https://www.ahrq.gov/sites/default/files/wysiwyg/research/findings/nhqrdr/2019qdr.pdf
  7. Hoffman KM, Trawalter S, Axt JR, et al. Racial bias in pain assessment and treatment recommendations, and false beliefs about biological differences between blacks and whites. Proc Natl Acad Sci U S A. 2016;113:4296-4301. doi:10.1073/pnas.1516047113
  8. Patel ZS, Hoffman LK, Buse DC, et al. Pain, psychological comorbidities, disability, and impaired quality of life in hidradenitis suppurativa. Curr Pain Headache Rep. 2017;21:49. doi:10.1007/s11916-017-0647-3. Published correction appears in Curr Pain Headache Rep. 2017;21:52.
  9. McDowell I. Pain measurements. In: Measuring Health: A Guide to Rating Scales and Questionnaires. Oxford University Press; 2006:477-478.
  10. Singh GK, Daus GP, Allender M, et al. Social determinants of health in the United States: addressing major health inequality trends for the nation, 1935-2016. Int J MCH AIDS. 2017;6:139-164. doi:10.21106/ijma.236
  11. Sulley S, Bayssie M. Social determinants of health: an evaluation of risk factors associated with inpatient presentations in the United States. Cureus. 2021;13:E13287. doi:10.7759/cureus.13287
  12. Lazar M, Davenport L. Barriers to health care access for low income families: a review of literature. J Community Health Nurs. 2018;35:28-37. doi:10.1080/07370016.2018.1404832
  13. Ghoshal M, Shapiro H, Todd K, et al. Chronic noncancer pain management and systemic racism: time to move toward equal care standards.J Pain Res. 2020;13:2825-2836. doi:10.214/JPR.S287314
  14. Cintron A, Morrison RS. Pain and ethnicity in the United States: a systematic review. J Palliat Med. 2006;9:1454-1473. doi:10.1089/jpm.2006.9.1454
  15. Green CR, Anderson KO, Baker TA, et al. The unequal burden of pain: confronting racial and ethnic disparities in pain. Pain Med. 2003;4:277-294. doi:10.1046/j.1526-4637.2003.03034.x. Published correction appears in Pain Med. 2005;6:99.
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  • Racial disparities exist in the management of hidradenitis suppurativa (HS)–related pain.
  • Black/African American patients with HS are 4 times more likely to experience very severe pain than patients of other races or ethnicities.
  • Lower income levels, higher HS disease severity, and a history of prescription pain medication use are all independent risk factors for very severe pain in patients with HS.
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Annular Plaques Overlying Hyperpigmented Telangiectatic Patches on the Neck

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Annular Plaques Overlying Hyperpigmented Telangiectatic Patches on the Neck

The Diagnosis: Annular Elastolytic Giant Cell Granuloma

Histologic examination of the shave biopsies showed a granulomatous infiltrate of small lymphocytes, histiocytes, and multinucleated giant cells. The giant cells have abundant eosinophilic cytoplasm, with several also containing fragments of basophilic elastic fibers (elastophagocytosis)(Figure). Additionally, the granulomas revealed no signs of necrosis, making an infectious source unlikely, and examination under polarized light was negative for foreign material. These clinical and histologic findings were diagnostic for annular elastolytic giant cell granuloma (AEGCG).

A biopsy showed basophilic elastic fibers in the cytoplasm of the multinucleated giant cells in the dermis (H&E, original magnifications ×400). Images courtesy of Anne Bussian, MD (Birmingham, Alabama).
A and B, A biopsy showed basophilic elastic fibers in the cytoplasm of the multinucleated giant cells in the dermis (H&E, original magnifications ×400). Images courtesy of Anne Bussian, MD (Birmingham, Alabama).

Annular elastolytic giant cell granuloma is a rare chronic inflammatory disorder that classically presents on sun-exposed skin as annular plaques with elevated borders and atrophic centers.1-4 Histologically, AEGCG is characterized by diffuse granulomatous infiltrates composed of multinucleated giant cells, histiocytes, and lymphocytes in the dermis, along with phagocytosis of elastic fibers by multinucleated giant cells.5 The underlying etiology and pathogenesis of AEGCG remains unknown.6

Annular elastolytic giant cell granuloma commonly affects females aged 35 to 75 years; however, cases have been reported in the male and pediatric patient populations.1,2 Documented cases are known to last from 1 month to 10 years.7,8 Although the mechanisms underlying the development of AEGCG remain to be elucidated, studies have determined that the skin disorder is associated with sarcoidosis, molluscum contagiosum, amyloidosis, diabetes mellitus, and cutaneous T-cell lymphoma.9 Diabetes mellitus is the most common comorbidity associated with AEGCG, and it is theorized that diabetes contributes to the increased incidence of AEGCG in this population by inducing damage to elastic fibers in the skin.10 One study that examined 50 cases of AEGCG found that 38 patients had serum glucose levels evaluated, with 8 cases being subsequently diagnosed with diabetes mellitus and 6 cases with apparent impaired glucose tolerance, indicating that 37% of the sample population with AEGCG who were evaluated for metabolic disease were found to have definitive or latent type 2 diabetes mellitus.11 Although AEGCG is a rare disorder, a substantial number of patients diagnosed with AEGCG also have diabetes mellitus, making it important to consider screening all patients with AEGCG for diabetes given the ease and widely available resources to check glucose levels.

Actinic granuloma, granuloma annulare, atypical facial necrobiosis lipoidica, granuloma multiforme, secondary syphilis, tinea corporis, and erythema annulare centrifugum most commonly are included in the differential diagnosis with AEGCG; histopathology is the key determinant in discerning between these conditions.12 Our patient presented with typical annular plaques overlying hyperpigmented telangiectatic patches. With known type 2 diabetes mellitus and the clinical findings, granuloma annulare, erythema annulare centrifugum, and AEGCG remained high on the differential.

No standard of care exists for AEGCG due to its rare nature and tendency to spontaneously resolve. The most common first-line treatment includes topical and intralesional steroids, topical pimecrolimus, and the use of sunscreen and other sun-protective measures. UV radiation, specifically UVA, has been determined to be a causal factor for AEGCG by changing the antigenicity of elastic fibers and producing an immune response in individuals with fair skin.13 Further, resistant cases of AEGCG successfully have been treated with cyclosporine, systemic steroids, antimalarials, dapsone, and oral retinoids.14,15 Some studies reported partial regression or full resolution with topical tretinoin; adalimumab; clobetasol ointment; or a combination of corticosteroids, antihistamines, and hydroxychloroquine.2 Only 1 case series using sulfasalazine reported worsening symptoms after treatment initiation.16 Our patient deferred systemic medications and was treated with 4 weeks of topical triamcinolone followed by 4 weeks of topical tacrolimus with minimal improvement. At the time of diagnosis, our patient also was encouraged to use sun-protective measures. At 6-month follow-up, the lesions remained stable, and the decision was made to continue with photoprotection.

References
  1. Mistry AM, Patel R, Mistry M, et al. Annular elastolytic giant cell granuloma. Cureus. 2020;12:E11456. doi:10.7759/cureus.11456
  2. Chen WT, Hsiao PF, Wu YH. Spectrum and clinical variants of giant cell elastolytic granuloma. Int J Dermatol. 2017;56:738-745. doi:10.1111/ijd.13502
  3. Raposo I, Mota F, Lobo I, et al. Annular elastolytic giant cell granuloma: a “visible” diagnosis. Dermatol Online J. 2017;23:13030/qt9rq3j927
  4. Klemke CD, Siebold D, Dippel E, et al. Generalised annular elastolytic giant cell granuloma. Dermatology. 2003;207:420-422. doi:10.1159/000074132
  5. Hassan R, Arunprasath P, Padmavathy L, et al. Annular elastolytic giant cell granuloma in association with Hashimoto’s thyroiditis. Indian Dermatol Online J. 2016;7:107-110. doi:10.4103/2229-5178.178087
  6. Kaya Erdog˘ an H, Arık D, Acer E, et al. Clinicopathological features of annular elastolytic giant cell granuloma patients. Turkish J Dermatol. 2018;12:85-89.
  7. Can B, Kavala M, Türkog˘ lu Z, et al. Successful treatment of annular elastolytic giant cell granuloma with hydroxychloroquine. Int J Dermatol. 2013;52:509-511. doi:10.1111 /j.1365-4632.2011.04941.x
  8. Arora S, Malik A, Patil C, et al. Annular elastolytic giant cell granuloma: a report of 10 cases. Indian Dermatol Online J. 2015;6(suppl 1):S17-S20. doi:10.4103/2229-5178.171055
  9. Doulaveri G, Tsagroni E, Giannadaki M, et al. Annular elastolytic giant cell granuloma in a 70-year-old woman. Int J Dermatol. 2003;42:290-291. doi:10.1046/j.1365-4362.2003.01767.x
  10. Marmon S, O’Reilly KE, Fischer M, et al. Papular variant of annular elastolytic giant-cell granuloma. Dermatol Online J. 2012;18:23.
  11. Aso Y, Izaki S, Teraki Y. Annular elastolytic giant cell granuloma associated with diabetes mellitus: a case report and review of the Japanese literature. Clin Exp Dermatol. 2011;36:917-919. doi:10.1111 /j.1365-2230.2011.04094.x
  12. Liu X, Zhang W, Liu Y, et al. A case of annular elastolytic giant cell granuloma associated with syphilis. Case Rep Dermatol. 2018; 10:158-161. doi:10.1159/000489910
  13. Gutiérrez-González E, Pereiro M Jr, Toribio J. Elastolytic actinic giant cell granuloma. Dermatol Clin. 2015;33:331-341. doi:10.1016/j.det.2015.03.002
  14. de Oliveira FL, de Barros Silveira LK, Machado Ade M, et al. Hybrid clinical and histopathological pattern in annular lesions: an overlap between annular elastolytic giant cell granuloma and granuloma annulare? Case Rep Dermatol Med. 2012;2012:102915. doi:10.1155/2012/102915
  15. Wagenseller A, Larocca C, Vashi NA. Treatment of annular elastolytic giant cell granuloma with topical tretinoin. J Drugs Dermatol. 2017;16:699-700.
  16. Yang YW, Lehrer MD, Mangold AR, et al. Treatment of granuloma annulare and related granulomatous diseases with sulphasalazine: a series of 16 cases. J Eur Acad Dermatol Venereol. 2021;35:211-215. doi:10.1111/jdv.16356
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Ms. Herron, Ms. Weir, and Dr. Kole are from the University of Alabama at Birmingham. Ms. Herron and Ms. Weir are from the Heersink School of Medicine, and Dr. Kole is from the Department of Dermatology. Dr. Herron is from Herron Dermatology and Laser, Montgomery, Alabama.

The authors report no conflict of interest.

Correspondence: Lauren C.S. Kole, MD, University of Alabama at Birmingham Heersink School of Medicine Department of Dermatology, 510 20th St S, Birmingham, AL 35233 ([email protected]).

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The authors report no conflict of interest.

Correspondence: Lauren C.S. Kole, MD, University of Alabama at Birmingham Heersink School of Medicine Department of Dermatology, 510 20th St S, Birmingham, AL 35233 ([email protected]).

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Ms. Herron, Ms. Weir, and Dr. Kole are from the University of Alabama at Birmingham. Ms. Herron and Ms. Weir are from the Heersink School of Medicine, and Dr. Kole is from the Department of Dermatology. Dr. Herron is from Herron Dermatology and Laser, Montgomery, Alabama.

The authors report no conflict of interest.

Correspondence: Lauren C.S. Kole, MD, University of Alabama at Birmingham Heersink School of Medicine Department of Dermatology, 510 20th St S, Birmingham, AL 35233 ([email protected]).

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The Diagnosis: Annular Elastolytic Giant Cell Granuloma

Histologic examination of the shave biopsies showed a granulomatous infiltrate of small lymphocytes, histiocytes, and multinucleated giant cells. The giant cells have abundant eosinophilic cytoplasm, with several also containing fragments of basophilic elastic fibers (elastophagocytosis)(Figure). Additionally, the granulomas revealed no signs of necrosis, making an infectious source unlikely, and examination under polarized light was negative for foreign material. These clinical and histologic findings were diagnostic for annular elastolytic giant cell granuloma (AEGCG).

A biopsy showed basophilic elastic fibers in the cytoplasm of the multinucleated giant cells in the dermis (H&E, original magnifications ×400). Images courtesy of Anne Bussian, MD (Birmingham, Alabama).
A and B, A biopsy showed basophilic elastic fibers in the cytoplasm of the multinucleated giant cells in the dermis (H&E, original magnifications ×400). Images courtesy of Anne Bussian, MD (Birmingham, Alabama).

Annular elastolytic giant cell granuloma is a rare chronic inflammatory disorder that classically presents on sun-exposed skin as annular plaques with elevated borders and atrophic centers.1-4 Histologically, AEGCG is characterized by diffuse granulomatous infiltrates composed of multinucleated giant cells, histiocytes, and lymphocytes in the dermis, along with phagocytosis of elastic fibers by multinucleated giant cells.5 The underlying etiology and pathogenesis of AEGCG remains unknown.6

Annular elastolytic giant cell granuloma commonly affects females aged 35 to 75 years; however, cases have been reported in the male and pediatric patient populations.1,2 Documented cases are known to last from 1 month to 10 years.7,8 Although the mechanisms underlying the development of AEGCG remain to be elucidated, studies have determined that the skin disorder is associated with sarcoidosis, molluscum contagiosum, amyloidosis, diabetes mellitus, and cutaneous T-cell lymphoma.9 Diabetes mellitus is the most common comorbidity associated with AEGCG, and it is theorized that diabetes contributes to the increased incidence of AEGCG in this population by inducing damage to elastic fibers in the skin.10 One study that examined 50 cases of AEGCG found that 38 patients had serum glucose levels evaluated, with 8 cases being subsequently diagnosed with diabetes mellitus and 6 cases with apparent impaired glucose tolerance, indicating that 37% of the sample population with AEGCG who were evaluated for metabolic disease were found to have definitive or latent type 2 diabetes mellitus.11 Although AEGCG is a rare disorder, a substantial number of patients diagnosed with AEGCG also have diabetes mellitus, making it important to consider screening all patients with AEGCG for diabetes given the ease and widely available resources to check glucose levels.

Actinic granuloma, granuloma annulare, atypical facial necrobiosis lipoidica, granuloma multiforme, secondary syphilis, tinea corporis, and erythema annulare centrifugum most commonly are included in the differential diagnosis with AEGCG; histopathology is the key determinant in discerning between these conditions.12 Our patient presented with typical annular plaques overlying hyperpigmented telangiectatic patches. With known type 2 diabetes mellitus and the clinical findings, granuloma annulare, erythema annulare centrifugum, and AEGCG remained high on the differential.

No standard of care exists for AEGCG due to its rare nature and tendency to spontaneously resolve. The most common first-line treatment includes topical and intralesional steroids, topical pimecrolimus, and the use of sunscreen and other sun-protective measures. UV radiation, specifically UVA, has been determined to be a causal factor for AEGCG by changing the antigenicity of elastic fibers and producing an immune response in individuals with fair skin.13 Further, resistant cases of AEGCG successfully have been treated with cyclosporine, systemic steroids, antimalarials, dapsone, and oral retinoids.14,15 Some studies reported partial regression or full resolution with topical tretinoin; adalimumab; clobetasol ointment; or a combination of corticosteroids, antihistamines, and hydroxychloroquine.2 Only 1 case series using sulfasalazine reported worsening symptoms after treatment initiation.16 Our patient deferred systemic medications and was treated with 4 weeks of topical triamcinolone followed by 4 weeks of topical tacrolimus with minimal improvement. At the time of diagnosis, our patient also was encouraged to use sun-protective measures. At 6-month follow-up, the lesions remained stable, and the decision was made to continue with photoprotection.

The Diagnosis: Annular Elastolytic Giant Cell Granuloma

Histologic examination of the shave biopsies showed a granulomatous infiltrate of small lymphocytes, histiocytes, and multinucleated giant cells. The giant cells have abundant eosinophilic cytoplasm, with several also containing fragments of basophilic elastic fibers (elastophagocytosis)(Figure). Additionally, the granulomas revealed no signs of necrosis, making an infectious source unlikely, and examination under polarized light was negative for foreign material. These clinical and histologic findings were diagnostic for annular elastolytic giant cell granuloma (AEGCG).

A biopsy showed basophilic elastic fibers in the cytoplasm of the multinucleated giant cells in the dermis (H&E, original magnifications ×400). Images courtesy of Anne Bussian, MD (Birmingham, Alabama).
A and B, A biopsy showed basophilic elastic fibers in the cytoplasm of the multinucleated giant cells in the dermis (H&E, original magnifications ×400). Images courtesy of Anne Bussian, MD (Birmingham, Alabama).

Annular elastolytic giant cell granuloma is a rare chronic inflammatory disorder that classically presents on sun-exposed skin as annular plaques with elevated borders and atrophic centers.1-4 Histologically, AEGCG is characterized by diffuse granulomatous infiltrates composed of multinucleated giant cells, histiocytes, and lymphocytes in the dermis, along with phagocytosis of elastic fibers by multinucleated giant cells.5 The underlying etiology and pathogenesis of AEGCG remains unknown.6

Annular elastolytic giant cell granuloma commonly affects females aged 35 to 75 years; however, cases have been reported in the male and pediatric patient populations.1,2 Documented cases are known to last from 1 month to 10 years.7,8 Although the mechanisms underlying the development of AEGCG remain to be elucidated, studies have determined that the skin disorder is associated with sarcoidosis, molluscum contagiosum, amyloidosis, diabetes mellitus, and cutaneous T-cell lymphoma.9 Diabetes mellitus is the most common comorbidity associated with AEGCG, and it is theorized that diabetes contributes to the increased incidence of AEGCG in this population by inducing damage to elastic fibers in the skin.10 One study that examined 50 cases of AEGCG found that 38 patients had serum glucose levels evaluated, with 8 cases being subsequently diagnosed with diabetes mellitus and 6 cases with apparent impaired glucose tolerance, indicating that 37% of the sample population with AEGCG who were evaluated for metabolic disease were found to have definitive or latent type 2 diabetes mellitus.11 Although AEGCG is a rare disorder, a substantial number of patients diagnosed with AEGCG also have diabetes mellitus, making it important to consider screening all patients with AEGCG for diabetes given the ease and widely available resources to check glucose levels.

Actinic granuloma, granuloma annulare, atypical facial necrobiosis lipoidica, granuloma multiforme, secondary syphilis, tinea corporis, and erythema annulare centrifugum most commonly are included in the differential diagnosis with AEGCG; histopathology is the key determinant in discerning between these conditions.12 Our patient presented with typical annular plaques overlying hyperpigmented telangiectatic patches. With known type 2 diabetes mellitus and the clinical findings, granuloma annulare, erythema annulare centrifugum, and AEGCG remained high on the differential.

No standard of care exists for AEGCG due to its rare nature and tendency to spontaneously resolve. The most common first-line treatment includes topical and intralesional steroids, topical pimecrolimus, and the use of sunscreen and other sun-protective measures. UV radiation, specifically UVA, has been determined to be a causal factor for AEGCG by changing the antigenicity of elastic fibers and producing an immune response in individuals with fair skin.13 Further, resistant cases of AEGCG successfully have been treated with cyclosporine, systemic steroids, antimalarials, dapsone, and oral retinoids.14,15 Some studies reported partial regression or full resolution with topical tretinoin; adalimumab; clobetasol ointment; or a combination of corticosteroids, antihistamines, and hydroxychloroquine.2 Only 1 case series using sulfasalazine reported worsening symptoms after treatment initiation.16 Our patient deferred systemic medications and was treated with 4 weeks of topical triamcinolone followed by 4 weeks of topical tacrolimus with minimal improvement. At the time of diagnosis, our patient also was encouraged to use sun-protective measures. At 6-month follow-up, the lesions remained stable, and the decision was made to continue with photoprotection.

References
  1. Mistry AM, Patel R, Mistry M, et al. Annular elastolytic giant cell granuloma. Cureus. 2020;12:E11456. doi:10.7759/cureus.11456
  2. Chen WT, Hsiao PF, Wu YH. Spectrum and clinical variants of giant cell elastolytic granuloma. Int J Dermatol. 2017;56:738-745. doi:10.1111/ijd.13502
  3. Raposo I, Mota F, Lobo I, et al. Annular elastolytic giant cell granuloma: a “visible” diagnosis. Dermatol Online J. 2017;23:13030/qt9rq3j927
  4. Klemke CD, Siebold D, Dippel E, et al. Generalised annular elastolytic giant cell granuloma. Dermatology. 2003;207:420-422. doi:10.1159/000074132
  5. Hassan R, Arunprasath P, Padmavathy L, et al. Annular elastolytic giant cell granuloma in association with Hashimoto’s thyroiditis. Indian Dermatol Online J. 2016;7:107-110. doi:10.4103/2229-5178.178087
  6. Kaya Erdog˘ an H, Arık D, Acer E, et al. Clinicopathological features of annular elastolytic giant cell granuloma patients. Turkish J Dermatol. 2018;12:85-89.
  7. Can B, Kavala M, Türkog˘ lu Z, et al. Successful treatment of annular elastolytic giant cell granuloma with hydroxychloroquine. Int J Dermatol. 2013;52:509-511. doi:10.1111 /j.1365-4632.2011.04941.x
  8. Arora S, Malik A, Patil C, et al. Annular elastolytic giant cell granuloma: a report of 10 cases. Indian Dermatol Online J. 2015;6(suppl 1):S17-S20. doi:10.4103/2229-5178.171055
  9. Doulaveri G, Tsagroni E, Giannadaki M, et al. Annular elastolytic giant cell granuloma in a 70-year-old woman. Int J Dermatol. 2003;42:290-291. doi:10.1046/j.1365-4362.2003.01767.x
  10. Marmon S, O’Reilly KE, Fischer M, et al. Papular variant of annular elastolytic giant-cell granuloma. Dermatol Online J. 2012;18:23.
  11. Aso Y, Izaki S, Teraki Y. Annular elastolytic giant cell granuloma associated with diabetes mellitus: a case report and review of the Japanese literature. Clin Exp Dermatol. 2011;36:917-919. doi:10.1111 /j.1365-2230.2011.04094.x
  12. Liu X, Zhang W, Liu Y, et al. A case of annular elastolytic giant cell granuloma associated with syphilis. Case Rep Dermatol. 2018; 10:158-161. doi:10.1159/000489910
  13. Gutiérrez-González E, Pereiro M Jr, Toribio J. Elastolytic actinic giant cell granuloma. Dermatol Clin. 2015;33:331-341. doi:10.1016/j.det.2015.03.002
  14. de Oliveira FL, de Barros Silveira LK, Machado Ade M, et al. Hybrid clinical and histopathological pattern in annular lesions: an overlap between annular elastolytic giant cell granuloma and granuloma annulare? Case Rep Dermatol Med. 2012;2012:102915. doi:10.1155/2012/102915
  15. Wagenseller A, Larocca C, Vashi NA. Treatment of annular elastolytic giant cell granuloma with topical tretinoin. J Drugs Dermatol. 2017;16:699-700.
  16. Yang YW, Lehrer MD, Mangold AR, et al. Treatment of granuloma annulare and related granulomatous diseases with sulphasalazine: a series of 16 cases. J Eur Acad Dermatol Venereol. 2021;35:211-215. doi:10.1111/jdv.16356
References
  1. Mistry AM, Patel R, Mistry M, et al. Annular elastolytic giant cell granuloma. Cureus. 2020;12:E11456. doi:10.7759/cureus.11456
  2. Chen WT, Hsiao PF, Wu YH. Spectrum and clinical variants of giant cell elastolytic granuloma. Int J Dermatol. 2017;56:738-745. doi:10.1111/ijd.13502
  3. Raposo I, Mota F, Lobo I, et al. Annular elastolytic giant cell granuloma: a “visible” diagnosis. Dermatol Online J. 2017;23:13030/qt9rq3j927
  4. Klemke CD, Siebold D, Dippel E, et al. Generalised annular elastolytic giant cell granuloma. Dermatology. 2003;207:420-422. doi:10.1159/000074132
  5. Hassan R, Arunprasath P, Padmavathy L, et al. Annular elastolytic giant cell granuloma in association with Hashimoto’s thyroiditis. Indian Dermatol Online J. 2016;7:107-110. doi:10.4103/2229-5178.178087
  6. Kaya Erdog˘ an H, Arık D, Acer E, et al. Clinicopathological features of annular elastolytic giant cell granuloma patients. Turkish J Dermatol. 2018;12:85-89.
  7. Can B, Kavala M, Türkog˘ lu Z, et al. Successful treatment of annular elastolytic giant cell granuloma with hydroxychloroquine. Int J Dermatol. 2013;52:509-511. doi:10.1111 /j.1365-4632.2011.04941.x
  8. Arora S, Malik A, Patil C, et al. Annular elastolytic giant cell granuloma: a report of 10 cases. Indian Dermatol Online J. 2015;6(suppl 1):S17-S20. doi:10.4103/2229-5178.171055
  9. Doulaveri G, Tsagroni E, Giannadaki M, et al. Annular elastolytic giant cell granuloma in a 70-year-old woman. Int J Dermatol. 2003;42:290-291. doi:10.1046/j.1365-4362.2003.01767.x
  10. Marmon S, O’Reilly KE, Fischer M, et al. Papular variant of annular elastolytic giant-cell granuloma. Dermatol Online J. 2012;18:23.
  11. Aso Y, Izaki S, Teraki Y. Annular elastolytic giant cell granuloma associated with diabetes mellitus: a case report and review of the Japanese literature. Clin Exp Dermatol. 2011;36:917-919. doi:10.1111 /j.1365-2230.2011.04094.x
  12. Liu X, Zhang W, Liu Y, et al. A case of annular elastolytic giant cell granuloma associated with syphilis. Case Rep Dermatol. 2018; 10:158-161. doi:10.1159/000489910
  13. Gutiérrez-González E, Pereiro M Jr, Toribio J. Elastolytic actinic giant cell granuloma. Dermatol Clin. 2015;33:331-341. doi:10.1016/j.det.2015.03.002
  14. de Oliveira FL, de Barros Silveira LK, Machado Ade M, et al. Hybrid clinical and histopathological pattern in annular lesions: an overlap between annular elastolytic giant cell granuloma and granuloma annulare? Case Rep Dermatol Med. 2012;2012:102915. doi:10.1155/2012/102915
  15. Wagenseller A, Larocca C, Vashi NA. Treatment of annular elastolytic giant cell granuloma with topical tretinoin. J Drugs Dermatol. 2017;16:699-700.
  16. Yang YW, Lehrer MD, Mangold AR, et al. Treatment of granuloma annulare and related granulomatous diseases with sulphasalazine: a series of 16 cases. J Eur Acad Dermatol Venereol. 2021;35:211-215. doi:10.1111/jdv.16356
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Annular Plaques Overlying Hyperpigmented Telangiectatic Patches on the Neck
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A 58-year-old man with a history of type 2 diabetes mellitus, nephrolithiasis, hypovitaminosis D, and hypercholesterolemia presented to our dermatology clinic for a follow-up total-body skin examination after a prior diagnosis of basal cell carcinoma on the vertex of the scalp. Physical examination revealed extensive photodamage and annular plaques overlying hyperpigmented telangiectatic patches on the dorsal portion of the neck. The eruption persisted for 1 year and failed to improve with clotrimazole cream. His medications included simvastatin, metformin, chlorthalidone, vitamin D, and tamsulosin. Two shave biopsies from the posterior neck were performed.

Annular plaques overlying hyperpigmented telangiectatic patches on the neck

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