Lichen Sclerosus et Atrophicus With Histopathologic Features Mimicking Mycosis Fungoides: A Large Series of Cases Comparing Genital With Extragenital Lichen Sclerosus. (January 2022)

• Record Type: Journal Article
• Authors: Leoni, Eleonora; Kempf, Werner; Cerroni, Lorenzo
• Is Part Of: American journal of surgical pathology. Volume 46:Number 1(2022)
• Publication Date: 2022-01
• Subjects: lichen sclerosus -- mycosis fungoides -- cutaneous T-cell pseudolymphoma -- cutaneous T-cell lymphoma -- cutaneous pseudolymphoma -- extragenital lichen sclerosus; Pathology, Surgical -- Periodicals; 617.0705
• DOI: 10.1097/PAS.0000000000001738
• Languages: English
• ISSNs: 0147-5185

Abstract

Abstract : Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory dermatosis of unknown etiology involving the genital and/or extragenital area, showing histopathologically a characteristic homogeneization and sclerosis of the superficial collagen with variably dense lymphoid infiltrates. Intraepidermal lymphocytes may be observed, and in some cases may pose differential diagnostic problems with mycosis fungoides (MF). We studied the histopathologic features of 121 cases of LSA with dense lymphoid infiltrates (genital: 94; male:female: 93:1; age range: 2 to 87 y; median age: 11 y; extragenital: 27; male:female: 0.1:1; age range: 11 to 79 y; median age: 59 y), to better characterize the intraepidermal lymphoid infiltrate and to compare genital with extragenital cases. Epidermotropic lymphocytes mimicking the histopathologic features of MF were present in 93.6% of the genital specimens but none of the extragenital cases. Interestingly, typical features of LSA were mssing in 39.4% of genital LSA, and in a further 25.5% were present only focally. Immunohistochemical analyses showed a predominance of CD8 + T-lymphocytes within the epidermis. Molecular studies of the T-cell receptor genes revealed a monoclonal population of T-lymphocytes in nearly half of the cases. Our study shows that MF-like histopathologic features are extremely common in genital LSA but are never encountered in extragenital cases. A diagnosis of MF in the genital area should be made only upon compelling features, keeping in mind the frequent pseudolymphomatous aspects of LSA.