Significant Disparities Persist in Cutaneous Presentations, Diagnosis, and Outcomes of Scleroderma in Skin of Color

Patients with skin of color and scleroderma, also known as systemic sclerosis (SSc), face significant disparities in diagnosis and outcomes due to varied cutaneous presentations, according to a review published in the Journal of the European Academy of Dermatology and Venereology.¹

Researchers hope that the study will prompt further studies with a goal of improving diagnostic and treatment challenges for this patient population.

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Background and Methods

SSc occurs more frequently in African American populations versus European Americans. To further complicate this disparity, it has also been documented that African American populations with SSc also face earlier average onset with a heightened risk of skin involvement and lung disease.²

Authors of the present review also noted that diagnosis and care of SSc may be hindered by clinicians' lack of recognition of the condition in darker skin types. Despite the prevalence of SSc in African American populations, the condition may be underdiagnosed or misdiagnosed in some cases.³

The systematic review involved a search of databases such as PubMed and SCOPUS for studies published between 2013 and 2023 on SSc in patients with skin of color. It included primary data studies that addressed SSc's presentation, diagnosis, and outcomes.

Findings

The review first included 82 initial studies, narrowed down to 9 relevant articles based on inclusion criteria and exclusion of duplicate materials. These studies varied in design, including cross-sectional, retrospective, and longitudinal cohorts, and focused on various ethnic groups including African American, African-Brazilian, and other skin of color populations.

The review studies reveal marked differences in SSc presentation among skin of color populations compared to White patients.

For instance, African American and African-Brazilian patients exhibited higher average modified Rodnan skin scores, indicating more severe cutaneous involvement. Additionally, African American patients had a higher prevalence of diffuse cutaneous disease (57%) compared to their White counterparts (37%). Diffuse skin involvement was also common among African-Brazilian patients, with similarly high rates of Raynaud's phenomenon and digital ulcers compared to White patients.

Several studies also highlighted ethnic variations in SSc manifestations. In the case of Afro-Caribbean patients, most individuals exhibited a higher incidence of diffuse cutaneous involvement and a greater prevalence of certain complications, such as lung disease.

Black patients in the EUSTAR database had significantly higher rates of diffuse skin involvement but lower prevalence of telangiectasias compared to White patients. Additionally, Black patients exhibited a higher frequency of severe lung disease and mortality rates.

One cross-sectional study highlighted the significant psychosocial impact of SSc on African American patients. Those with skin of color experienced greater body image disturbances and dissatisfaction related to digital ulcers, pigmentation changes, and pruritus.

Conclusions

Researchers noted that this study adds to and highlights disparities faced by patients with scleroderma and skin of color. Limitations of the review may include potential bias of reported data and subjective differences in defining skin of color.

"Varied clinical manifestations, such as atypical cutaneous symptoms, antibody profiles and disease severity, emphasize the complexity of diagnosing SSc in SOC populations," according to Podwojniak et al. "However, many uncertainties remain and are deserving of further research."

Moving forward, review authors highlighted the necessity of future studies to expand upon this research. They noted that given patients with skin of color often experience more severe clinical manifestations of SSc, accompanied by a greater risk of disease-related complications, clinicians and researchers should keep these factors in mind when treating and conducting research for patients with skin of color.

References

1. Podwojniak A, Tan IJ, Leiter A, Ghani H, Cohen BA. Cutaneous presentations of systemic sclerosis in skin of colour: a systematic review. J Eur Acad Dermatol Venereol. August 15, 2024. https://doi.org/10.1002/jvc2.527
2. National Institute of Arthritis and Musculoskeletal and Skin Diseases. Scleroderma. National Institute of Arthritis and Musculoskeletal and Skin Diseases. Accessed August 21, 2024. https://www.niams.nih.gov/health-topics/scleroderma#:~:text=The%20disease%20is%20more%20common,when%20compared%20with%20other%20groups
3. Shanmugavadivel D, Liu JF, Buonsenso D, Davis T, Roland D. Assessing healthcare professionals’ identification of paediatric dermatological conditions in darker skin tones. Children. 2022; 9(11):1749. https://doi.org/10.3390/children9111749