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Clinical, Cosmetic and Investigational... 2024Restricted pagetoid reticulosis, also known as Woringer-Kolopp disease, represents a rare cutaneous lymphoproliferative disorder categorized as an isolated variant of...
Restricted pagetoid reticulosis, also known as Woringer-Kolopp disease, represents a rare cutaneous lymphoproliferative disorder categorized as an isolated variant of mycosis fungoides. This report presents a case involving limited pagetoid reticulosis affecting the right upper extremity in a 25-year-old female. The patient had been experiencing plaques on the right upper extremity for a decade. Dermatologic examination revealed well-defined scaly plaques on the right forearm, surrounded by hyperpigmented patches. Skin histopathology demonstrated atypical mononuclear cell infiltration in the lower part of the epidermis, forming nests. Immunohistochemistry indicated CD3+, CD4+, CD5+, CD7+, CD8+, CD30+, and Ki-67-positive staining. Additionally, CD20, CD79α, and PD-1 were negative. Monoclonal rearrangement of T-cells was identified in TCR β and TCR γ through clonality assessment. The diagnosis of limited paget-like reticulocyte hyperplasia was established, leading to surgical resection. A review of the literature affirmed the variable immunophenotype of pagetoid reticulosis, with atypical cells exhibiting four types: (1) CD3+, CD4+, CD8+-type; (2) CD3+, CD4-, CD8+-type; (3) CD3+, CD4-, CD8-type; and (4) CD3+, CD4+, CD8+-type-relatively uncommon in the restrictive type. This case report details the clinical features, histologic and morphologic characteristics, immunohistochemical phenotype, diagnosis, and differential diagnosis of a rare CD3+, CD4+, CD8+ limited pagetoid reticulosis. The lesion was surgically resected, and the patient underwent a 3-year follow-up to observe its prognosis.
PubMed: 38827631
DOI: 10.2147/CCID.S462966 -
Frontiers in Oncology 2023Mycosis fungoides (MF) and Sézary syndrome (SS) are cutaneous T-cell lymphomas. MF is the most common cutaneous lymphoma, and it is classified into classic... (Review)
Review
Mycosis fungoides (MF) and Sézary syndrome (SS) are cutaneous T-cell lymphomas. MF is the most common cutaneous lymphoma, and it is classified into classic Alibert-Bazin MF, folliculotropic MF, pagetoid reticulosis, and granulomatous slack skin, each with characteristic clinical presentation, histopathological findings, and distinct clinical behaviors. SS is an aggressive leukemic variant of cutaneous lymphoma, and it is characterized by erythroderma, lymphadenopathy, and peripheral blood involvement by malignant cells. There is a wide range of dermatological manifestations of MF/SS, and prompt recognition is essential for early diagnosis. Skin biopsy for histopathology and immunohistochemical analysis is imperative to confirm the diagnosis of MF/SS. Histopathology may also provide information that may influence prognosis and treatment. Staging follows the TNMB system. Besides advanced stage, other factors associated with poorer prognosis are advanced age, male gender, folliculotropism in histopathology of patients with infiltrated plaques and tumors in the head and neck region, large cell transformation, and elevated lactate dehydrogenase. Treatment is divided into skin-directed therapies (topical treatments, phototherapy, radiotherapy), and systemic therapies (biological response modifiers, targeted therapies, chemotherapy). Allogeneic bone marrow transplantation and extracorporeal photopheresis are other treatment modalities used in selected cases. This review discusses the main clinical characteristics, the histopathological/immunohistochemical findings, the staging system, and the therapeutic management of MF/SS.
PubMed: 37124514
DOI: 10.3389/fonc.2023.1141108 -
Healthcare (Basel, Switzerland) Feb 2023Mycosis fungoides is the most common primary cutaneous T-cell lymphoma, characterized by skin-homing CD4+ T cells derivation, indolent course, and low-grade of... (Review)
Review
Mycosis fungoides is the most common primary cutaneous T-cell lymphoma, characterized by skin-homing CD4+ T cells derivation, indolent course, and low-grade of malignancy. Mycosis fungoides's classic type typically onsets with cutaneous erythematous patches, plaque, and tumor. In WHO-EORTC classification, folliculotropic mycosis fungoides, pagetoid reticulosis, and granulomatous slack skin are recognized as distinct variants of mycosis fungoides, because of their clinical and histological features, behavior, and /or prognosis. Mycosis fungoides often shows diagnostic difficulties, due to its absence of specific features and lesional polymorphism. A patient's treatment requires staging. In about 10% of cases, mycosis fungoides can progress to lymph nodes and internal organs. Prognosis is poor at advanced stage and management needs a multidisciplinary team approach. Advanced stage disease including tumors, erythroderma, and nodal, visceral, or blood involvement needs skin directed therapy associated with systemic drugs. Skin directed therapy includes steroids, nitrogen mustard, bexarotene gel, phototherapy UVB, and photochemiotherapy, i.e., total skin electron radiotherapy. Systemic therapies include retinoids, bexarotene, interferon, histone deacetylase inhibitors, photopheresis, targeted immunotherapy, and cytotoxic chemotherapy. Complexity of mycosis fungoides associated with long-term chronic evolution and multiple therapy based on disease stage need a multidisciplinary team approach to be treated.
PubMed: 36833148
DOI: 10.3390/healthcare11040614 -
Romanian Journal of Morphology and... 2021Neoplasms with pagetoid features are a category of rare lesions defined by the presence of atypical cells at different levels of the epidermis. The most important...
INTRODUCTION
Neoplasms with pagetoid features are a category of rare lesions defined by the presence of atypical cells at different levels of the epidermis. The most important diseases within this category are mammary Paget disease (MPD), extramammary Paget disease (EMPD), Bowen's disease, in situ melanoma, and pagetoid reticulosis.
AIM
The aim of this analysis was to describe the importance of the cytokeratin 8∕18 (CK 8∕18) immunostaining in diagnosing MPD and EMPD and differentiating them from other lesions.
MATERIALS AND METHODS
A retrospective study was employed, based on the histopathological and immunohistochemical (IHC) characteristics of 30 cases that presented pagetoid features. The cases were processed and analyzed at the Department of Pathology, Mureş Clinical County Hospital, Târgu Mureş, Romania, from 2017 to 2020.
RESULTS
Five MPD cases, one EMPD case, one pagetoid reticulosis case, 10 Bowen's disease cases, and 13 in situ melanoma cases were collected. Under Hematoxylin-Eosin staining, cells presented pale cytoplasm in MPD, EMPD, and in 25% of the melanoma cases. Hyperchromasia with nuclear enlargement was seen in all cases. Immunostaining with CK 8∕18 was positive in all MPD and EMPD cases. Tests for CK7, p63, and CK AE1∕AE3 were positive in MPD, EMPD, and Bowen's cases. Tests for S100, SRY-box transcription factor 10 (SOX10), human melanoma black 45 (HMB45), and Melan A were positive in melanoma cases, while cluster of differentiation (CD)3, CD4, and CD8 tests were positive in the pagetoid reticulosis case.
CONCLUSIONS
CK 8∕18 is an IHC marker that can help establish the diagnosis of MPD and EMPD and differentiate them from other pagetoid neoplasms, ensuring the proper diagnosis and prognosis are provided.
Topics: Biomarkers; Biomarkers, Tumor; Bowen's Disease; Diagnosis, Differential; Humans; Keratin-18; Keratin-8; Retrospective Studies; Skin Neoplasms
PubMed: 35263404
DOI: 10.47162/RJME.62.3.13 -
Dermatology Practical & Conceptual Feb 2022
PubMed: 35223183
DOI: 10.5826/dpc.1201a39 -
Cureus Jan 2022Background Mycosis fungoides (MF) is one of the primary cutaneous T-cell lymphomas and is considered to be the most common extranodal non-Hodgkin lymphomas. MF is...
Background Mycosis fungoides (MF) is one of the primary cutaneous T-cell lymphomas and is considered to be the most common extranodal non-Hodgkin lymphomas. MF is characterized by different subtypes based on clinical presentation and immunophenotyping studies. We aimed to study the clinical patterns and treatment response in cases of MF among the patients attending a tertiary referral hospital in Saudi Arabia. Methodology A retrospective study, case record-based study was done to review all the patients diagnosed with MF from January 2011 to May 2016. All cases with histopathological confirmation and immunophenotyping were included in the study. Treatment follow-up was reviewed for 9 months in all cases. Treatment response was graded based on a global physician assessment-complete response, good response, moderate response, and minimal or no response. Results Out of 34 cases of MF included in the study, 11 were hyperpigmented MF, 21 were hypopigmented MF, and there was one case each of poikilodermatous MF and pagetoid reticulosis. Of the total, fourteen (66.7%) of hypopigmented MF patients showed a complete response to phototherapy Narrowband UVB (NB-UVB) in combination with topical corticosteroids. Nine (81.8%) of hyperpigmented MF patients showed partial to the phototherapy NB-UVB in combination with the topical corticosteroid. Among the other types; one case of poikilodermatous MF (2.9%) showed a moderate response to phototherapy NB-UVB with topical corticosteroid and systemic acitretin. Conclusions The most common type of MF seen in our study was the hypopigmented type, affecting a younger age group, and the same showed a good response to phototherapy NB-UVB combined with topical corticosteroids.
PubMed: 35174031
DOI: 10.7759/cureus.21231 -
Cureus Oct 2021Pagetoid reticulosis is a rare form of cutaneous T cell lymphoma, a malignancy of T lymphocytes, that invades the skin leading to a multitude of dermatologic...
Pagetoid reticulosis is a rare form of cutaneous T cell lymphoma, a malignancy of T lymphocytes, that invades the skin leading to a multitude of dermatologic manifestations. Pagetoid reticulosis commonly presents as a localized slow-growing or indolently manifesting hyperkeratotic patch/plaque on the extremities that is confined to the epidermis. Diagnosis is confirmed via a skin biopsy of the affected area followed by a cytologic examination. Treatment typically entails topical corticosteroids, alkylating agents, and retinoids. With disseminated disease, a multidisciplinary approach involving chemotherapy and radiation is necessary. To raise awareness about pagetoid reticulosis, its diagnosis, and management, we report a case localized to the left forehead and earlobe of a 57-year-old female.
PubMed: 34754679
DOI: 10.7759/cureus.18524 -
JAAD Case Reports Jan 2019
PubMed: 30581947
DOI: 10.1016/j.jdcr.2018.10.023 -
Dermatopathology (Basel, Switzerland) 2016Pagetoid reticulosis is a rare variant of mycosis fungoides that presents with a large, usually single, erythematous, slowly growing scaly plaque containing an...
Pagetoid reticulosis is a rare variant of mycosis fungoides that presents with a large, usually single, erythematous, slowly growing scaly plaque containing an intraepidermal proliferation of neoplastic T lymphocytes. Histopathologically, this disease has distinctive attributes. In this report, we present two cases of pagetoid reticulosis, compare its microscopic features to those of 'classical' mycosis fungoides, and provide a brief review of the pertinent literature.
PubMed: 27195265
DOI: 10.1159/000444660