Lymphomatoid Papulosis Type E With T-Cell Receptor Gamma Positivity

Amornrut Namasondhi,1 Suthinee Rutnin,1 Suthep Jerasutus,1 Paisarn Boonsakan,2 Korn Triyangkulsri1 1Division of Dermatology, Department of Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand; 2Department of Pathology, Faculty of Medicine, Ramathibodi Hospital,...

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Main Authors: Namasondhi A, Rutnin S, Jerasutus S, Boonsakan P, Triyangkulsri K
Format: Article
Language:English
Published: Dove Medical Press 2025-01-01
Series:Clinical, Cosmetic and Investigational Dermatology
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Online Access:https://www.dovepress.com/lymphomatoid-papulosis-type-e-with-t-cell-receptor-gamma-positivity-peer-reviewed-fulltext-article-CCID
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author Namasondhi A
Rutnin S
Jerasutus S
Boonsakan P
Triyangkulsri K
author_facet Namasondhi A
Rutnin S
Jerasutus S
Boonsakan P
Triyangkulsri K
author_sort Namasondhi A
collection DOAJ
description Amornrut Namasondhi,1 Suthinee Rutnin,1 Suthep Jerasutus,1 Paisarn Boonsakan,2 Korn Triyangkulsri1 1Division of Dermatology, Department of Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand; 2Department of Pathology, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, ThailandCorrespondence: Korn Triyangkulsri, Division of Dermatology, Department of Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, 270 Rama VI Road, Ratchathewi, Bangkok, 10400, Thailand, Tel +66-2-2011141, Fax +66-2-201-1211, Email korn.triy@gmail.comAbstract: Lymphomatoid papulosis (LyP) is currently categorized as a primary lymphoproliferative disorder that follows a chronic, recurrent clinical course. The diagnosis of LyP is mainly based on clinical presentation and histopathological correlation. Six subtypes of LyP have been described and recognized, each with different histological features and sometimes distinct clinical presentations. LyP type E is a subtype that histologically shows angioinvasion and angiodestruction by CD8 and CD30-positive pleomorphic T cells. Clinically, it usually presents with a few large necrotic nodules or ulcers on the trunk or extremities, unlike other subtypes of LyP. Despite an indolent clinical course, long-term follow-up is necessary due to the risk of developing concurrent or secondary lymphoma. In this report, we demonstrate a case of lymphomatoid papulosis type E presented with widespread small papulonecrotic eruptions, an atypical clinical manifestation, and an unusual immunohistochemical profile. The biopsy revealed CD8, CD30, CD56, and TCR-γ-positive atypical lymphocytic infiltration with angioinvasion and angiodestruction. The patient was successfully treated with low-dose methotrexate.Keywords: CD30, CD56, cutaneous T-cell lymphoma, lymphoproliferative disorder, papulonecrotic skin disease
format Article
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institution Kabale University
issn 1178-7015
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publishDate 2025-01-01
publisher Dove Medical Press
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series Clinical, Cosmetic and Investigational Dermatology
spelling doaj-art-4d487226ce4a485aa951c132491334ca2025-01-21T16:58:06ZengDove Medical PressClinical, Cosmetic and Investigational Dermatology1178-70152025-01-01Volume 1817718299378Lymphomatoid Papulosis Type E With T-Cell Receptor Gamma PositivityNamasondhi ARutnin SJerasutus SBoonsakan PTriyangkulsri KAmornrut Namasondhi,1 Suthinee Rutnin,1 Suthep Jerasutus,1 Paisarn Boonsakan,2 Korn Triyangkulsri1 1Division of Dermatology, Department of Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand; 2Department of Pathology, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, ThailandCorrespondence: Korn Triyangkulsri, Division of Dermatology, Department of Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, 270 Rama VI Road, Ratchathewi, Bangkok, 10400, Thailand, Tel +66-2-2011141, Fax +66-2-201-1211, Email korn.triy@gmail.comAbstract: Lymphomatoid papulosis (LyP) is currently categorized as a primary lymphoproliferative disorder that follows a chronic, recurrent clinical course. The diagnosis of LyP is mainly based on clinical presentation and histopathological correlation. Six subtypes of LyP have been described and recognized, each with different histological features and sometimes distinct clinical presentations. LyP type E is a subtype that histologically shows angioinvasion and angiodestruction by CD8 and CD30-positive pleomorphic T cells. Clinically, it usually presents with a few large necrotic nodules or ulcers on the trunk or extremities, unlike other subtypes of LyP. Despite an indolent clinical course, long-term follow-up is necessary due to the risk of developing concurrent or secondary lymphoma. In this report, we demonstrate a case of lymphomatoid papulosis type E presented with widespread small papulonecrotic eruptions, an atypical clinical manifestation, and an unusual immunohistochemical profile. The biopsy revealed CD8, CD30, CD56, and TCR-γ-positive atypical lymphocytic infiltration with angioinvasion and angiodestruction. The patient was successfully treated with low-dose methotrexate.Keywords: CD30, CD56, cutaneous T-cell lymphoma, lymphoproliferative disorder, papulonecrotic skin diseasehttps://www.dovepress.com/lymphomatoid-papulosis-type-e-with-t-cell-receptor-gamma-positivity-peer-reviewed-fulltext-article-CCIDcd30cd56cutaneous t-cell lymphomalymphoproliferative disorderpapulonecrotic skin disease
spellingShingle Namasondhi A
Rutnin S
Jerasutus S
Boonsakan P
Triyangkulsri K
Lymphomatoid Papulosis Type E With T-Cell Receptor Gamma Positivity
Clinical, Cosmetic and Investigational Dermatology
cd30
cd56
cutaneous t-cell lymphoma
lymphoproliferative disorder
papulonecrotic skin disease
title Lymphomatoid Papulosis Type E With T-Cell Receptor Gamma Positivity
title_full Lymphomatoid Papulosis Type E With T-Cell Receptor Gamma Positivity
title_fullStr Lymphomatoid Papulosis Type E With T-Cell Receptor Gamma Positivity
title_full_unstemmed Lymphomatoid Papulosis Type E With T-Cell Receptor Gamma Positivity
title_short Lymphomatoid Papulosis Type E With T-Cell Receptor Gamma Positivity
title_sort lymphomatoid papulosis type e with t cell receptor gamma positivity
topic cd30
cd56
cutaneous t-cell lymphoma
lymphoproliferative disorder
papulonecrotic skin disease
url https://www.dovepress.com/lymphomatoid-papulosis-type-e-with-t-cell-receptor-gamma-positivity-peer-reviewed-fulltext-article-CCID
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AT rutnins lymphomatoidpapulosistypeewithtcellreceptorgammapositivity
AT jerasutuss lymphomatoidpapulosistypeewithtcellreceptorgammapositivity
AT boonsakanp lymphomatoidpapulosistypeewithtcellreceptorgammapositivity
AT triyangkulsrik lymphomatoidpapulosistypeewithtcellreceptorgammapositivity