Background: There are divergent opinions whether atrophoderma Pasini-Pierini (APP) is a nosologic entity or a primary atrophic morphea. Objective: Since usually single cases are reported without a long-term follow-up the present study was performed in order to elucidate the natural history of the disorder. Methods: We followed a large series of 139 patients, 91 adults and 48 children, for 4–30 years (mean over 10 years). Results: APP was found to be 6 times more frequent in females and not uncommon in children (10% of our series of localized scleroderma). At some time during the follow-up period, indurations appeared in the central parts of the lesions in 17% of the patients, and in 22% they coexisted with morphea plaques outside the atrophies. The histological pattern was similar to morphea at the stage of atrophy. No case developed full-blown morphea. Conclusion: APP appears to be an abortive morphea, in which the indurations failed to develop. The differentiation from morphea is of practical importance because of different management and prognosis.

Our clinical and immunological studies of 114 cases of systemic sclerosis, 54 of Raynaud's disease and 46 of other connective tissue diseases, centered on the diagnostic and prognostic significance of anticentromere antibodies (ACA). The ACA occurred in 21 of 84 patients with acrosclerosis, in four of 54 patients with Raynaud's disease but in none of 30 patients with diffuse scleroderma or transitional form, acrosclerosis-diffuse scleroderma, or 46 cases of other connective tissue diseases. The ACA-positive patients had no contracture or immobilization of the fingers, the indurations and/or indurative oedema were confined to fingers and usually no other types of ANA were detected. However, systemic involvement and the course of the disease were comparable in ACA-negative and ACA-positive acrosclerosis patients. The studies indicate that there is a subset of acrosclerosis with minimal indurations confined to the fingers, and ACA appears to be its serological marker. We propose to use the term CREST for this subset, which to date has not been exactly defined and is regarded by some authors as synonymous with acrosclerosis.

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