From Localized Scleroderma to Systemic Sclerosis: Coexistence or Possible Evolution

Table 1

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

Number	Age/sex	First diagnosis	Clinical picture at the 1st rheumatologic visit	Time to 2nd disease onset (months)	Second diagnosis	Clinical picture at the 2nd diagnosis	Other SSc features during the follow-up
(1)	26 F	LoS	Morphea at right leg from 2 years; RP onset 4 years before, new telangiectasias, nondiagnostic alterations at VC, ANoA with ENA neg.	24	SSc	RP, sclerodactyly and sclerodermic face, ACA plus anti-SSA, DU, “early” SSc pattern at VC	Esophagopathy
(2)	60 F	LoS	Morphea at the abdomen from 2 years, ANoA, nonspecific pattern at VC	48	SSc	ANoA, “active” SSc pattern at VC, Esophagopathy	RP, sclerodactyly and sclerodermic face, ILD
(3)	33 F	LoS	Recent onset of morphea at right arm and face, ANA speckled, SSc pattern at VC, 2 episodes of RP	7	SSc	RP, puffy hands, ANA speckled, “early” SSc pattern at VC	- (pregnancy complicated by IUGR)
(4)	69 F	LoS	Recent onset of morphea at dorsum, previous RP, puffy hands, Scl70, DU, “active” SSc pattern at VC	contemporary	SSc	-	Sclerodermic face
(5)	50 F	LoS	Recent onset of morphea at trunk and right thigh, doubtful very mild sclerodactyly, ANA speckled, aspecific pattern at VC	12	SSc	RP, sclerodactyly, ANoA, aspecific pattern at VC	Esophagopathy
(6)	83 F	LoS	Morphea at dorsum from 13 years, RP, ACA	156	SSc	RP, mild sclerodactyly, ACA, “early” SSc pattern at VC, sicca syndrome, DLCO 68%	DLCO further reduction (56%)
(7)	70 F	SSc	RP, sclerodactyly, ACA, DU, Esophagopathy	120	LoS	Left pretibial linear LoS	-
(8)	50 F	SSc	RP, sclerodactyly, ACA, DU, “early” SSc pattern at VC, melanodermia, calcinosis	60	LoS	Morphea 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.