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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
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.
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.