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Morphea Profunda with Tertiary Lymphoid Follicles: Description of Two Cases and Review of the Literature
Angelo Cassisa1, Margherita Vannucchi2
1Section of Pathology, Department of Oncology, San Giovanni di Dio Hospital, USL Centro Toscana, 50143 Florence, Italy.
Abstract:
Morphea profunda or subcutaneous (deep) morphea is a variant of localized morphea, characterized by one or more ill-defined, deep sclerotic plaque. Preferential sites are the abdomen, trunk, sacral area, or extremities. The presence of hyperplastic lymphoid follicles in the context of the sclerotic bands of morphea is rarely described. Localized scleroderma is sustained by a profibrotic inflammatory profile. Transforming growth factor-β (TGF-β), an imbalance between functional subclasses of T-lymphocytes (innate immune cells) has a role in activate collagen deposition. In this case report, we present two cases of morphea profunda with lymphoid follicular hyperplasia. A systematic review of the literature on the pathophysiology of localized scleroderma is also presented, with particular reference to the presence of lymphoid structures.
Insights
Morphea profunda, a deep form of localized scleroderma, can present with rare lymphoid follicular hyperplasia. This case report highlights this association and reviews the underlying pathophysiology of scleroderma.
Area of Science:
- Dermatology
- Immunology
- Pathology
Background:
- Morphea profunda is a deep variant of localized scleroderma, presenting as ill-defined sclerotic plaques.
- Localized scleroderma involves a profibrotic inflammatory profile, with transforming growth factor-beta (TGF-β) and T-lymphocyte imbalances contributing to collagen deposition.
Observation:
- This case report details two instances of morphea profunda exhibiting lymphoid follicular hyperplasia.
- This specific finding, lymphoid follicular hyperplasia within sclerotic bands, is infrequently documented in morphea profunda.
Findings:
- The study observed a rare association between morphea profunda and lymphoid follicular hyperplasia.
- A systematic literature review on localized scleroderma pathophysiology, focusing on lymphoid structures, accompanies these case reports.
Implications:
- Understanding the role of lymphoid structures in morphea profunda may offer new insights into localized scleroderma pathogenesis.
- Further research into the immunopathology of morphea profunda could guide future therapeutic strategies.

