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

Research output: Contribution to journalArticlepeer-review


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 languageEnglish (US)
Pages (from-to)475-481
Number of pages7
JournalJournal of Rheumatology
Issue number5
StatePublished - May 1 2022


  • hypopigmentation
  • perifollicular
  • pigmentation
  • scleroderma
  • systemic sclerosis

ASJC Scopus subject areas

  • Immunology and Allergy
  • Rheumatology
  • Immunology


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