Morphea Profunda with Tertiary Lymphoid Follicles: Description of Two Cases and Review of the Literature

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.

Systemic sclerosis with morphea-like plaques histopathologically mimicking cutaneous B-cell lymphoma

“…Yet, even in inflammatory morphea, lymphoid follicles have only rarely been reported. 3 In a recent review of 137 cases of morphea, the lymphocytic infiltrates were variably distributed in patterns that were perivascular or periadnexal or at the dermal-subcutaneous interface. Lymphoid follicles are not specifically described in this large case series.…”

“…Lymphoid follicles are not a typical feature but have been rarely reported in association with dermal sclerosis in skin biopsies of morphea. 3 , 4 We report a case of systemic sclerosis presenting with morphea-like plaques and prominent lymphoid follicles on skin biopsy. These findings were misdiagnosed initially as cutaneous B-cell lymphoma.…”