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

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.