Perifollicular Hypopigmentation in Systemic Sclerosis: Associations With Clinical Features and Internal Organ Involvement

Melody P. Chung; Christopher A. Mecoli; Jamie Perin; Carrie Richardson; Zsuzsanna H. McMahan

doi:10.3899/jrheum.210983

Perifollicular Hypopigmentation in Systemic Sclerosis: Associations With Clinical Features and Internal Organ Involvement

Melody P. Chung, Christopher A. Mecoli, Jamie Perin, Carrie Richardson, Zsuzsanna H. McMahan^*

^*Corresponding author for this work

Medicine, Rheumatology Division

Research output: Contribution to journal › Article › peer-review

3 Scopus citations

Abstract

Objective. To determine whether perifollicular hypopigmentation in systemic sclerosis (SSc) is associated with demographics, distinct clinical features, and autoantibody profiles. Methods. Patients with SSc were prospectively enrolled, with a standardized data form used to collect anatomic distribution of perifollicular hypopigmentation. Associations between hypopigmentation and features of SSc were assessed. Results. Of 179 adult patients with SSc, 36 (20%) patients had perifollicular hypopigmentation. Of these 36 patients, 94% (n = 34) were female and 33% (n = 12) had limited cutaneous SSc. In univariable logistic regression, Black race (odds ratio [OR] 15.63, 95% CI 6.6–37.20, P < 0.001), diffuse cutaneous SSc (dcSSc; OR 4.62, 95% CI 2.11–10.09, P < 0.001), higher maximum modified Rodnan skin score (mRSS; OR 1.05, 95% CI 1.02–1.08, P = 0.003), myopathy (OR 3.92, 95% CI 1.80–8.57, P < 0.001), pulmonary fibrosis (OR 2.69, 95% CI 1.20-6.02, P = 0.02), lower minimum forced vital capacity % predicted (OR 0.96, 95% CI 0.94–0.99, P = 0.001), and lower minimum diffusing capacity for carbon monoxide % predicted (OR 0.97, 95% CI 0.95–0.99, P = 0.009) were associated with hypopigmentation. Anticentromere antibodies inversely associated with hypopigmentation (OR 0.24, 95% CI 0.07–0.86, P = 0.03). After adjusting for age, race, and disease duration, dcSSc (OR 4.28, 95% CI 1.46-12.53, P = 0.008) and increased mRSS (OR 1.07, 95% CI 1.02–1.12, P = 0.009) were significantly associated with hypopigmentation. Conclusion. Perifollicular hypopigmentation is observed in a subset of patients with SSc and associated with diffuse subtype. Larger prospective studies determining whether perifollicular hypopigmentation precedes end-organ involvement and whether specific patterns associate with internal organ involvement are needed.

Original language	English (US)
Pages (from-to)	475-481
Number of pages	7
Journal	Journal of Rheumatology
Volume	49
Issue number	5
DOIs	https://doi.org/10.3899/jrheum.210983
State	Published - May 1 2022

Funding

This study was supported by the National Institutes of Health/National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIH/NIAMS) K23 AR071473, Scleroderma Research Foundation, and Jerome L. Greene Foundation (ZHM); and NIH/NIAMS K23 AR075898 (CAM). The Cresanthe Stauraluakis Memorial Discovery Fund supported the autoantibody assays. 1M.P. Chung, MD, MS, C.A. Mecoli, MD, MHS, Z.H. McMahan, MD, MHS, Division of Rheumatology, Johns Hopkins University, Baltimore, Maryland; 2J. Perin, PhD, MS, Institute for International Programs, Johns Hopkins Bloomberg School of Public Health, Baltimore, Maryland; 3C. Richardson, MD, MHS, Division of Rheumatology, Rush University, Chicago, Illinois, USA. The authors declare no conflicts of interest relevant to this article. Address correspondence to Dr. Z.H. McMahan, Associate Professor of Medicine, Division of Rheumatology, Johns Hopkins University, 5200 Eastern Avenue, Mason F. Lord Building, Center Tower, Suite 4100, Baltimore, MD 21224, USA. Email: [email protected]. Accepted for publication January 25, 2022.

Keywords

hypopigmentation
perifollicular
pigmentation
scleroderma
systemic sclerosis

ASJC Scopus subject areas

Immunology and Allergy
Rheumatology
Immunology

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10.3899/jrheum.210983

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@article{bf69d0c4ad364d67b7d02078559cafb9,

title = "Perifollicular Hypopigmentation in Systemic Sclerosis: Associations With Clinical Features and Internal Organ Involvement",

abstract = "Objective. To determine whether perifollicular hypopigmentation in systemic sclerosis (SSc) is associated with demographics, distinct clinical features, and autoantibody profiles. Methods. Patients with SSc were prospectively enrolled, with a standardized data form used to collect anatomic distribution of perifollicular hypopigmentation. Associations between hypopigmentation and features of SSc were assessed. Results. Of 179 adult patients with SSc, 36 (20%) patients had perifollicular hypopigmentation. Of these 36 patients, 94% (n = 34) were female and 33% (n = 12) had limited cutaneous SSc. In univariable logistic regression, Black race (odds ratio [OR] 15.63, 95% CI 6.6–37.20, P < 0.001), diffuse cutaneous SSc (dcSSc; OR 4.62, 95% CI 2.11–10.09, P < 0.001), higher maximum modified Rodnan skin score (mRSS; OR 1.05, 95% CI 1.02–1.08, P = 0.003), myopathy (OR 3.92, 95% CI 1.80–8.57, P < 0.001), pulmonary fibrosis (OR 2.69, 95% CI 1.20-6.02, P = 0.02), lower minimum forced vital capacity % predicted (OR 0.96, 95% CI 0.94–0.99, P = 0.001), and lower minimum diffusing capacity for carbon monoxide % predicted (OR 0.97, 95% CI 0.95–0.99, P = 0.009) were associated with hypopigmentation. Anticentromere antibodies inversely associated with hypopigmentation (OR 0.24, 95% CI 0.07–0.86, P = 0.03). After adjusting for age, race, and disease duration, dcSSc (OR 4.28, 95% CI 1.46-12.53, P = 0.008) and increased mRSS (OR 1.07, 95% CI 1.02–1.12, P = 0.009) were significantly associated with hypopigmentation. Conclusion. Perifollicular hypopigmentation is observed in a subset of patients with SSc and associated with diffuse subtype. Larger prospective studies determining whether perifollicular hypopigmentation precedes end-organ involvement and whether specific patterns associate with internal organ involvement are needed.",

keywords = "hypopigmentation, perifollicular, pigmentation, scleroderma, systemic sclerosis",

author = "Chung, {Melody P.} and Mecoli, {Christopher A.} and Jamie Perin and Carrie Richardson and McMahan, {Zsuzsanna H.}",

note = "Funding Information: This study was supported by the National Institutes of Health/National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIH/NIAMS) K23 AR071473, Scleroderma Research Foundation, and Jerome L. Greene Foundation (ZHM); and NIH/NIAMS K23 AR075898 (CAM). The Cresanthe Stauraluakis Memorial Discovery Fund supported the autoantibody assays. 1M.P. Chung, MD, MS, C.A. Mecoli, MD, MHS, Z.H. McMahan, MD, MHS, Division of Rheumatology, Johns Hopkins University, Baltimore, Maryland; 2J. Perin, PhD, MS, Institute for International Programs, Johns Hopkins Bloomberg School of Public Health, Baltimore, Maryland; 3C. Richardson, MD, MHS, Division of Rheumatology, Rush University, Chicago, Illinois, USA. The authors declare no conflicts of interest relevant to this article. Address correspondence to Dr. Z.H. McMahan, Associate Professor of Medicine, Division of Rheumatology, Johns Hopkins University, 5200 Eastern Avenue, Mason F. Lord Building, Center Tower, Suite 4100, Baltimore, MD 21224, USA. Email: [email protected]. Accepted for publication January 25, 2022. Publisher Copyright: {\textcopyright} 2022 The Journal of Rheumatology.",

year = "2022",

month = may,

day = "1",

doi = "10.3899/jrheum.210983",

language = "English (US)",

volume = "49",

pages = "475--481",

journal = "Journal of Rheumatology",

issn = "0315-162X",

publisher = "Journal of Rheumatology",

number = "5",

}

TY - JOUR

T1 - Perifollicular Hypopigmentation in Systemic Sclerosis

T2 - Associations With Clinical Features and Internal Organ Involvement

AU - Chung, Melody P.

AU - Mecoli, Christopher A.

AU - Perin, Jamie

AU - Richardson, Carrie

AU - McMahan, Zsuzsanna H.

N1 - Funding Information: This study was supported by the National Institutes of Health/National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIH/NIAMS) K23 AR071473, Scleroderma Research Foundation, and Jerome L. Greene Foundation (ZHM); and NIH/NIAMS K23 AR075898 (CAM). The Cresanthe Stauraluakis Memorial Discovery Fund supported the autoantibody assays. 1M.P. Chung, MD, MS, C.A. Mecoli, MD, MHS, Z.H. McMahan, MD, MHS, Division of Rheumatology, Johns Hopkins University, Baltimore, Maryland; 2J. Perin, PhD, MS, Institute for International Programs, Johns Hopkins Bloomberg School of Public Health, Baltimore, Maryland; 3C. Richardson, MD, MHS, Division of Rheumatology, Rush University, Chicago, Illinois, USA. The authors declare no conflicts of interest relevant to this article. Address correspondence to Dr. Z.H. McMahan, Associate Professor of Medicine, Division of Rheumatology, Johns Hopkins University, 5200 Eastern Avenue, Mason F. Lord Building, Center Tower, Suite 4100, Baltimore, MD 21224, USA. Email: [email protected]. Accepted for publication January 25, 2022. Publisher Copyright: © 2022 The Journal of Rheumatology.

PY - 2022/5/1

Y1 - 2022/5/1

N2 - Objective. To determine whether perifollicular hypopigmentation in systemic sclerosis (SSc) is associated with demographics, distinct clinical features, and autoantibody profiles. Methods. Patients with SSc were prospectively enrolled, with a standardized data form used to collect anatomic distribution of perifollicular hypopigmentation. Associations between hypopigmentation and features of SSc were assessed. Results. Of 179 adult patients with SSc, 36 (20%) patients had perifollicular hypopigmentation. Of these 36 patients, 94% (n = 34) were female and 33% (n = 12) had limited cutaneous SSc. In univariable logistic regression, Black race (odds ratio [OR] 15.63, 95% CI 6.6–37.20, P < 0.001), diffuse cutaneous SSc (dcSSc; OR 4.62, 95% CI 2.11–10.09, P < 0.001), higher maximum modified Rodnan skin score (mRSS; OR 1.05, 95% CI 1.02–1.08, P = 0.003), myopathy (OR 3.92, 95% CI 1.80–8.57, P < 0.001), pulmonary fibrosis (OR 2.69, 95% CI 1.20-6.02, P = 0.02), lower minimum forced vital capacity % predicted (OR 0.96, 95% CI 0.94–0.99, P = 0.001), and lower minimum diffusing capacity for carbon monoxide % predicted (OR 0.97, 95% CI 0.95–0.99, P = 0.009) were associated with hypopigmentation. Anticentromere antibodies inversely associated with hypopigmentation (OR 0.24, 95% CI 0.07–0.86, P = 0.03). After adjusting for age, race, and disease duration, dcSSc (OR 4.28, 95% CI 1.46-12.53, P = 0.008) and increased mRSS (OR 1.07, 95% CI 1.02–1.12, P = 0.009) were significantly associated with hypopigmentation. Conclusion. Perifollicular hypopigmentation is observed in a subset of patients with SSc and associated with diffuse subtype. Larger prospective studies determining whether perifollicular hypopigmentation precedes end-organ involvement and whether specific patterns associate with internal organ involvement are needed.

AB - Objective. To determine whether perifollicular hypopigmentation in systemic sclerosis (SSc) is associated with demographics, distinct clinical features, and autoantibody profiles. Methods. Patients with SSc were prospectively enrolled, with a standardized data form used to collect anatomic distribution of perifollicular hypopigmentation. Associations between hypopigmentation and features of SSc were assessed. Results. Of 179 adult patients with SSc, 36 (20%) patients had perifollicular hypopigmentation. Of these 36 patients, 94% (n = 34) were female and 33% (n = 12) had limited cutaneous SSc. In univariable logistic regression, Black race (odds ratio [OR] 15.63, 95% CI 6.6–37.20, P < 0.001), diffuse cutaneous SSc (dcSSc; OR 4.62, 95% CI 2.11–10.09, P < 0.001), higher maximum modified Rodnan skin score (mRSS; OR 1.05, 95% CI 1.02–1.08, P = 0.003), myopathy (OR 3.92, 95% CI 1.80–8.57, P < 0.001), pulmonary fibrosis (OR 2.69, 95% CI 1.20-6.02, P = 0.02), lower minimum forced vital capacity % predicted (OR 0.96, 95% CI 0.94–0.99, P = 0.001), and lower minimum diffusing capacity for carbon monoxide % predicted (OR 0.97, 95% CI 0.95–0.99, P = 0.009) were associated with hypopigmentation. Anticentromere antibodies inversely associated with hypopigmentation (OR 0.24, 95% CI 0.07–0.86, P = 0.03). After adjusting for age, race, and disease duration, dcSSc (OR 4.28, 95% CI 1.46-12.53, P = 0.008) and increased mRSS (OR 1.07, 95% CI 1.02–1.12, P = 0.009) were significantly associated with hypopigmentation. Conclusion. Perifollicular hypopigmentation is observed in a subset of patients with SSc and associated with diffuse subtype. Larger prospective studies determining whether perifollicular hypopigmentation precedes end-organ involvement and whether specific patterns associate with internal organ involvement are needed.

KW - hypopigmentation

KW - perifollicular

KW - pigmentation

KW - scleroderma

KW - systemic sclerosis

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Perifollicular Hypopigmentation in Systemic Sclerosis: Associations With Clinical Features and Internal Organ Involvement

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