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Atrophoderma Pasini-Pierini is a primary atrophic abortive morphea

D Kencka1, M Blaszczyk, S Jabłońska

  • 1Department of Dermatology, Warsaw School of Medicine, Poland.

Dermatology (Basel, Switzerland)
|January 1, 1995
PubMed
Summary

Atrophoderma Pasini-Pierini (APP) is likely an abortive form of morphea, lacking indurations. This distinction is crucial for patient management and prognosis, especially given its occurrence in children.

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Area of Science:

  • Dermatology
  • Autoimmune diseases
  • Scleroderma spectrum disorders

Background:

  • Atrophoderma Pasini-Pierini (APP) diagnostic classification remains debated, with differing views on whether it is a distinct entity or a variant of primary atrophic morphea.
  • Limited long-term follow-up data exists for APP, hindering a clear understanding of its natural history.

Purpose of the Study:

  • To investigate the natural history of Atrophoderma Pasini-Pierini (APP).
  • To clarify the relationship between APP and morphea through long-term patient observation.

Main Methods:

  • A longitudinal study was conducted on 139 patients diagnosed with APP (91 adults, 48 children).
  • Patient follow-up ranged from 4 to 30 years, with a mean duration exceeding 10 years.

Main Results:

  • APP demonstrated a higher prevalence in females (6:1 ratio) and was observed in 10% of pediatric localized scleroderma cases.
  • During follow-up, 17% of patients developed central indurations within lesions, and 22% showed coexisting morphea plaques.
  • Histological findings were consistent with morphea in the atrophic stage; however, no patients progressed to fully developed morphea.

Conclusions:

  • Atrophoderma Pasini-Pierini (APP) is proposed to be an abortive form of morphea, characterized by the absence of induration development.
  • Distinguishing APP from morphea is clinically significant due to differing treatment strategies and prognostic outlooks.

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