Localized Lichen Myxedematosus With Plasma Cell Light Chain Restriction: Is It the Exception or the Rule?
Autor: | Yvana Cespedes, Angela Combs, Laszlo J. Karai, Lacey B. Elwyn, Georgette Rodriguez-Vazquez, Evangelos Poulos, Maher Albitar, Gabriel Villada |
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Rok vydání: | 2019 |
Předmět: |
Adult
Male Pathology medicine.medical_specialty Systemic disease Biopsy Plasma Cells Dermatology Plasma cell Pathology and Forensic Medicine Pathogenesis 030207 dermatology & venereal diseases 03 medical and health sciences Lichen myxedematosus Immunoglobulin kappa-Chains Young Adult 0302 clinical medicine Localized lichen myxedematosus Immunoglobulin lambda-Chains Scleromyxedema medicine Humans In Situ Hybridization Skin business.industry General Medicine medicine.disease Mucinosis stomatognathic diseases medicine.anatomical_structure Paraproteins business Facial Dermatoses |
Zdroj: | The American Journal of dermatopathology. 41(7) |
ISSN: | 1533-0311 |
Popis: | Lichen myxedematosus is a chronic cutaneous mucinosis that can present on a spectrum from localized cutaneous lesions to systemic disease of scleromyxedema. The clinical presentation of localized cutaneous lichen myxedematosus is waxy lichenoid papules, nodules, and/or plaques that have histopathologic findings of mucin deposition and a variable degree of fibroblast proliferation. There is an absence of serum paraproteins, and there are no other systemic causes of cutaneous mucinosis such as thyroid disease. The pathogenesis of lichen myxedematosus is unknown. We report 3 cases of localized cutaneous lichen myxedematosus with a light chain-restricted plasmacytic component by in situ hybridization. Our findings deliver an insight for disease pathogenesis and highlight for the first time, the significance of plasma cells in lesions of localized cutaneous lichen myxedematosus. We suggest that plasma cell light chain restriction could represent a clue to distinguish localized cutaneous disease from systemic disease. |
Databáze: | OpenAIRE |
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