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Updated: May 22, 2026
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Clinicopathological study of 81 cases of localized and systemic scleroderma
F Succaria1, M Kurban, A-G Kibbi
1Department of Dermatology, American University of Beirut Medical Center, Lebanon.
Background:
Scleroderma is a connective tissue disease that includes localized and systemic forms. Our recent encounter with a morphea case exhibiting prominent perineural inflammation microscopically prompted us to assess the features of all patients diagnosed with morphea/scleroderma at our institution.
Objective/Methods:
To describe the clinicopathological features of all patients diagnosed with morphea/scleroderma at American University of Beirut Medical Center (AUB-MC) between 1999 and 2010, and compare our findings with those published in the literature.
Results:
A total of 81 cases (63 women and 18 men) were identified, of which 73 were localized (morphea) and eight were systemic scleroderma. Clinically, plaque type morphea was the most common variant both in adults and children, and seven (9%) cases of morphea were associated with lichen sclerosis et atrophicus (LSA). Histopathologically, perineural inflammation was observed in 49% of cases, and may serve, in addition to other features including lichen sclerosis-like changes (observed in exclusively nine cases of morphea), more diffuse dermal and less subcutaneous sclerosis, and intense inflammation, as clues favouring diagnosis of morphea over systemic sclerosis.
Conclusion:
The features of morphea/scleroderma patients in this study are generally comparable to those published in the literature, with few differences. Clinically, plaque type morphea was the most common variant both in adults and children and LSA was a frequent association. Histopathologically, perineural inflammation was commonly observed and may serve in addition to lichen sclerosis-like changes and intense inflammation as clues favouring diagnosis of morphea over systemic sclerosis.
Insights
This study analyzed 81 scleroderma cases, finding plaque morphea most common. Perineural inflammation was frequent, aiding morphea diagnosis over systemic sclerosis.
Area of Science:
- Dermatology
- Rheumatology
- Pathology
Background:
- Scleroderma encompasses localized and systemic connective tissue diseases.
- Morphea, a localized form, can present with significant perineural inflammation.
- This study investigated clinicopathological features of morphea/scleroderma patients.
Purpose of the Study:
- To describe clinicopathological features of morphea/scleroderma patients.
- To compare findings with existing literature.
- To identify diagnostic clues differentiating morphea from systemic sclerosis.
Main Methods:
- Retrospective analysis of 81 morphea/scleroderma cases.
- Clinicopathological data collected between 1999 and 2010.
- Comparison of institutional findings with published literature.
Main Results:
- 73 localized (morphea) and 8 systemic scleroderma cases identified.
- Plaque morphea was the most common clinical variant in adults and children.
- Perineural inflammation observed in 49% of cases; lichen sclerosis et atrophicus (LSA) in 9%.
Conclusions:
- Morphea/scleroderma features align with literature, with notable associations.
- Plaque morphea and LSA association are clinically significant.
- Perineural inflammation, LSA-like changes, and intense inflammation aid morphea diagnosis.
