title: Clinical utility of hypo- and hyperpigmentation of skin in diffuse cutaneous systemic sclerosis.
abstract: OBJECTIVE
  Cutaneous involvement is an early manifestation of systemic sclerosis (SSc). Localized areas of 'salt and pepper skin' (S&P) may develop. We hypothesize that S&P skin occurs frequently in diffuse cutaneous (dc) SSc which can be used in its early diagnosis and may correlate with joint contractures.
  
  
  METHODS
  Sixty-five patients were recruited for this study. The demographic profiles of SSc were ascertained from hospital records. These patients fulfilled the 2013 American College of Rheumatology/European League Against Rheumatism classification criteria. Patients were examined for skin pigmentary changes, modified Rodnan skin score (mRSS), telengiectasias, calcinosis, arthritis and joint contractures and pruritus.
  
  
  RESULTS
  Sixty-five patients (59 female) were recruited with median age of 62.87 years. Forty-four had limited cutaneous SSc, 16 dcSSc, five had scleroderma overlap syndrome. Multivariate stepwise logistic regression indicated that mRSS severity and the presence of contractures were independently (P < 0.05) associated with dcSSc. The strong positive association between S&P and mRSS severity may explain the non-significance of S&P in this analysis. If mRSS severity is not included in the logistic regression analysis, the presence of contractures and S&P (odds ratio = 15.1) show significant (P < 0.01) independent associations with the dcSSc subtype. S&P skin and pruritus were similar in patients with Scl-70 and anti-RNA polymerase antibodies. Anti-centromere antibodies were negatively associated with S&P (χ2= 7.89, P = 0.005).
  
  
  CONCLUSIONS
  Our study demonstrates strong association of S&P skin with dcSSc (69%), increased risk of pruritus and contractures. Its presence can be used as another clinical tool to diagnose dcSSc in early stages. Observing for S&P skin changes does not require much training.
authors.affiliation: Rheumatology Department, Waikato Hospital, Hamilton, New Zealand.|Waikato Clinical School, University of Auckland, Auckland, New Zealand.
authors.forename: Kamal K
authors.initials: KK
authors.lastname: Solanki
authors.affiliation: Western Hospital, Melbourne, Victoria, Australia.
authors.forename: Cecil
authors.initials: C
authors.lastname: Hor
authors.affiliation: Ng Teng Fong Hospital, Jurong, Singapore.
authors.forename: Winston S J
authors.initials: WSJ
authors.lastname: Chang
authors.affiliation: Dunedin University, Dunedin, New Zealand.
authors.forename: Christopher
authors.initials: C
authors.lastname: Frampton
authors.affiliation: Rheumatology Department, Waikato Hospital, Hamilton, New Zealand.|Waikato Clinical School, University of Auckland, Auckland, New Zealand.
authors.forename: Douglas H N
authors.initials: DHN
authors.lastname: White
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D016009:Chi-Square Distribution; D042241:Early Diagnosis; D005260:Female; D006801:Humans; D017495:Hyperpigmentation / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D017496:Hypopigmentation / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D016015:Logistic Models; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D016017:Odds Ratio; D011237:Predictive Value of Tests; D011379:Prognosis; D011537:Pruritus / Q000209:etiology; D045743:Scleroderma, Diffuse / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D012720:Severity of Illness Index; D012880:Skin Pigmentation
keywords: calcinosis; clinical aspects systemic sclerosis; joint contractures; pruritus; salt and pepper skin