Atrophoderma Pasini-Pierini is a primary atrophic abortive morphea.

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.