Dermatology Research and Practice / 2018 / Article / Tab 1

Research Article

From Localized Scleroderma to Systemic Sclerosis: Coexistence or Possible Evolution

Table 1

Summary of the patients of our series with LoS associated with SSc.

NumberAge/sexFirst diagnosisClinical picture at the 1st rheumatologic visitTime to 2nd disease onset (months)Second diagnosisClinical picture at the 2nd diagnosisOther SSc features during the follow-up

(1)26 FLoSMorphea at right leg from 2 years; RP onset 4 years before, new telangiectasias, nondiagnostic alterations at VC, ANoA with ENA neg.24SScRP, sclerodactyly and sclerodermic face, ACA plus anti-SSA, DU, “early” SSc pattern at VCEsophagopathy

(2)60 FLoSMorphea at the abdomen from 2 years, ANoA, nonspecific pattern at VC48SScANoA, “active” SSc pattern at VC, EsophagopathyRP, sclerodactyly and sclerodermic face, ILD

(3)33 FLoSRecent onset of morphea at right arm and face, ANA speckled, SSc pattern at VC, 2 episodes of RP7SScRP, puffy hands, ANA speckled, “early” SSc pattern at VC- (pregnancy complicated by IUGR)

(4)69 FLoSRecent onset of morphea at dorsum, previous RP, puffy hands, Scl70, DU, “active” SSc pattern at VCcontemporarySSc-Sclerodermic face

(5)50 FLoSRecent onset of morphea at trunk and right thigh, doubtful very mild sclerodactyly, ANA speckled, aspecific pattern at VC12SScRP, sclerodactyly, ANoA, aspecific pattern at VCEsophagopathy

(6)83 FLoSMorphea at dorsum from 13 years, RP, ACA156SScRP, mild sclerodactyly, ACA, “early” SSc pattern at VC, sicca syndrome, DLCO 68%DLCO further reduction (56%)

(7)70 FSScRP, sclerodactyly, ACA, DU, Esophagopathy120LoSLeft pretibial linear LoS-

(8)50 FSScRP, sclerodactyly, ACA, DU, “early” SSc pattern at VC, melanodermia, calcinosis60LoSMorphea at trunk-

Legend. In the first 6 cases LoS was the first diagnosis made by a dermatologist; successively, these patients referred to ourRheumatology Unit because of the suspect of an unrecognized SSc. After a variable period, SSc diagnosis was formulated in presence of a SSc-specific clinical picture. During the follow-ups, eventual new features of the disease appeared; in the 7th and 8th case (italic rows) Los developed in the course of a definite SSc, in patients referring to our Rheumatology Unit. The second diagnosis (LoS) was confirmed by the dermatologist.

Article of the Year Award: Outstanding research contributions of 2020, as selected by our Chief Editors. Read the winning articles.