Abstract:

Cui Xiangxiang, Yang Ji, Tu Wenzhen, Li Ming Department of Dermatology, Children′s Hospital of Shanghai, Shanghai 200062, China （Cui XX）; Department of Dermatology, Zhongshan Hospital, Fudan University, Shanghai 200032, China （Yang J, Li M）; Department of Scleroderma, Shanghai TCM-integrated Hospital, shanghai 200082, China （Tu WZ） Corresponding author: Li Ming, Email: li.ming@zs-hospital.sh.cn 【Abstract】 Objective To investigate features of skin lesions of patients with systemic sclerosis （SSc）, and to analyze the relationship of skin manifestations with clinical classification, autoantibodies and internal organ involvement. Methods Clinical data were collected from 120 patients with SSc in Department of Dermatology of Zhongshan Hospital Affiliated to Fudan University and Department of Scleroderma of Shanghai TCM-integrated Hospital between 2012 and 2014, and analyzed retrospectively. Results Among the 120 patients with SSc, 118 （98.3%） had Raynaud′s phenomenon, 116 （96.7%） had skin sclerosis, including 101（84.2%） with sclerosis of skin over the dorsum of fingers, 90 （75%） had swollen skin, including 84（70%）with swollen fingers, 77 （64.2%） had poikiloderma, 75 （62.5%） had thinned lip, 74 （61.7%） had telangiectasia, 63 （52.5%） had radial furrowing around the mouth, 57 （47.5%）had mask-like face, 49（40.8%） had hyperplasia of nail cuticle, 35 （29.2%） had petechiae of the nailfolds, 25 （20.8%） had depressed fingertip, 24 （20.0%） had atrophy of the finger pulp, 24（20.0%） had distal finger shortening, and 15 （12.5%） had fingertip ulcer. Anti-Scl-70 antibody and anti-centromere antibody （ACA） were positive in 42 （35.0%） and 31 （25.8%） patients respectively. The incidence of swollen fingers, fingertip ulcer, atrophy of the finger pulp was significantly higher in the anti-Scl-70 antibody-positive group than in the -negative group（P < 0.05）, and the incidence of sclerosis of skin over the dorsum of fingers, poikiloderma, fingertip ulcer and atrophy of the finger pulp was all significantly higher in the anti-Scl-70 antibody-positive group than in the ACA-positive group（P < 0.05）. The main internal organ involvement included interstitial lung disease （50%, 44/88）, cardiac involvement （47.8%, 55/115）, pulmonary arterial hypertension （35.7%, 41/115）, esophageal involvement （28.3%, 34/120） and kidney involvement （9.2%, 11/120）. Patients with diffuse cutaneous systemic sclerosis （dcSSc） showed significantly higher incidence of cardiac involvement and poikiloderma compared with those with limited cutaneous systemic sclerosis （lcSSc）（P < 0.01）. Swollen fingers, sclerosis of skin over the dorsum of fingers, poikiloderma, telangiectasia, lip thinning, and radial furrowing around the mouth most commonly occurred in patients with early SSc, and swollen fingers and sclerosis of skin over the dorsum of fingers were highly correlated with the occurrence of pulmonary arterial hypertension. Telangiectasia, depressed fingertip and fingertip ulcer were significantly correlated with the occurrence of interstitial lung disease, and atrophy of the finger pulp was significantly correlated with the occurrence of cardiac involvement （both P < 0.05）. Conclusions Raynaud′s phenomenon, swollen fingers, sclerosis of skin over the dorsum of fingers, poikiloderma, telangiectasia, lip thinning and radial furrowing around the mouth can be helpful for the early diagnosis of SSc. Pulmonary arterial hypertension commonly occurs in the early stage of SSc. Depressed fingertip and fingertip ulcer indicate the occurrence of interstitial lung disease, and the atrophy of the finger pulp indicates cardiac involvement.