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.

[Studies of the role of cellular immunity and angiogenetic changes in the pathogenesis of circumscribed scleroderma]. / Badania nad rola odczynowosci komórkowej i zmian naczyniowych w patogenezie twardziny ograniczonej.

The studies were performed in 48 patients with morphea and included evaluation of 1) antinuclear antibodies 2) lymphocyte induced angiogenesis 3) natural killer (NK) cell activity and 4) T cell subpopulations in peripheral blood. The presence of antinuclear antibodies was found in 44.4% (8/18) patients with scleroderma linearis and in 21% (4/19) patients with morphea disseminata. Lymphocyte induced angiogenesis was increased in 41.5% (17/41) morphea patients, mainly in cases with pronounced vascular changes. The E rosette forming test showed a decreased percentage of active rosette forming cells (ARFC) and total rosette forming cells (TRFC) in peripheral blood and the NK cell activity was lowered in patients with morphea. These results obtained in patients with morphea show some similarities and differences in comparison to cellular immunity disturbances in patients with systemic scleroderma.

Anticentromere antibody: an immunological marker of a subset of systemic sclerosis.

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.

[Fasciitis eosinophilica--transition to an unusual scleroderma (scleroderma-fasciitis)]. / Fasciitis Eosinophilica--Ubergang in eine eigenartige Sklerodermie (Sklerodermie - Fasciitis).

Eosionophilic fasciitis (EF) was observed in a girl who suddenly developed indurations of the limbs accompanied by hypergammaglobulinaemia and peripheral eosinophilia. The deep fascia was impressively thickened and infiltrated with numerous inflammatory cells showing focal accumulations of eosinophils. The disease was progressive in spite of application of corticosteroids, and within three years, symmetrical sclerodermatous lesions developed on the face and trunk, going along with a severe sclerodactyly without Raynaud's phenomenon, as well as contractures of the upper and lower limbs causing complete disability. Visceral involvement was slight (decreased motility of the esophagus and restriction of pulmonal function); slight calcium deposits were disclosed at the soft tissue of the ankles; and nuclear antibodies of the speckled type appeared in a titer up to 320. The fascia was still the most involved tissue, but there was no peripheral or tissue eosinophilia. EF seems to be a special variant within the broad spectrum of scleroderma; and although it usually has a benign course, it may change into an unclassified variety of scleroderma and lead to severe disability.