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[ZKKL] Academic Presentation | Recent Advances in the Classification and Treatment of Lymphomatoid Papulosis
Release time:2019-11-01
Reprinted from: Chinese Journal of Dermatology and Venereology, Issue 05, 2017, p. 557.
Authors: Li Yuan, Ma Cuiling, Wang Lei
[Abstract] Lymphomatoid papulosis (lymphomatoid pap-ulosis, Ly P) It is a relatively common low-grade malignant cutaneous lymphoma, and together with primary cutaneous anaplastic large-cell lymphoma, it belongs to the category of cutaneous… CD30 Positive lymphoproliferative disorder. Ly P Clinically, it presents as chronic, relapsing, self‑healing papules and nodules; pathologically, it is characterized by the proliferation of atypical lymphocytes within the epidermis or dermis, with tumor cells expressing… CD30 Marking. This disease is caused by Ma-caulay at 1968 It was first named in [year], but its origin was not yet clear at the time. 1972 Year Black Waiting in Ly P Abundant hydrolases were detected in the atypical large cells, leading to the erroneous conclusion that this disease originates from histiocytes. 1981 Year Weinman Wait and Brehmer-Andersson Then respectively propose Ly P It originates from lymphocytes. 1986 Year Weiss Wait for approval TC The method for detecting R gene rearrangements has confirmed that… Ly P middle T The monoclonal nature of cells, in the same year Kaudewitz Once confirmed by immunohistochemistry, Ly P Tumor cells in express CD30 . This article briefly introduces Ly P The classification of its subtypes, along with its distinctive clinical, pathological, and immunohistochemical features, highlights the remaining diagnostic challenges, and briefly outlines treatment strategies for lymphomatoid papulosis.
[Keywords] Lymphomatoid Papulosis 、 virus 、 Disease
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Basic Classification of Ly P
1. 1 A Type and B Type 1982 Willemze Wait for the… Ly P Divided into A Type and B Type. A The histopathological type is the most common; it is characterized by wedge-shaped infiltrates composed of scattered or clustered atypical anaplastic large cells intermixed with small lymphocytes, neutrophils, and eosinophils. Because the tumor cells resemble histiocytes in morphology, this subtype was also referred to as the histiocytic type in earlier literature. B This type is a rare subtype, characterized by involvement of the dermis. CD4 Positive small to medium-sized pleomorphic cells show wedge-shaped infiltration or band-like epidermotropic infiltration; the tumor cells resemble gyral lymphocytes seen in mycosis fungoides.
1. 2 C Type 1993 Willemze Cases that initially present clinically as necrotic papules or nodules, yet exhibit pathological features resembling anaplastic large-cell lymphoma, are defined as a new entity. Ly P Subtype. Its clinical features are consistent with classic lymphomatoid papulosis, but the pathology reveals patchy infiltration of atypical large cells within the dermis, resembling cutaneous anaplastic large-cell lymphoma.
1. 3 D Type 2010 Saggini Wait for the report. 9 Example patients, whose clinical characteristics are consistent with… Ly P Consistent with an inert biological behavior. However, the pathological findings demonstrate a marked tropism for the epidermis. CD8+ Lymphocytic infiltration, with small to medium-sized lymphocytes, resembling the primary cutaneous invasive epidermotropic type. CD8 The pathology of positive cytotoxic lymphoma is similar, but the tumor cells exhibit varying degrees of expression. CD30 Marking. In China, Xiong Jingshu and Wen Pengfei, among others, have reported similar cases. 2012 year Kempf Wait until the report is submitted. 13 In cases similar to this patient, the authors, however, diagnosed it as expression. CD30 Acute pityriasis lichenoides et varioliformis acuta ( Acute pityriasis lichenoides et varioliformis acuta , PLEVA) . Recent research has found that approximately 1 /3 of children Ly P All patients had previously suffered from… PLEVA Alternatively, it may be chronic lichenoid pityriasis. To date, more than… have been reported. 15 For example, in patients with lichenoid pityriasis, lesions have been found to contain… CD30 + Cellular infiltration, and the study found that in some cases… PLEVA A monoclonal population can be detected. T Cells. These findings suggest that D Type Ly P With and PLEVA An absolute distinction between them is not possible; the two may be considered a spectrum of disorders.
1. 4 E Type 2012 Kempf Wait until the first report was filed. 16 Example And E Type Ly P The patient typically presents with solitary or a few scattered papules that rapidly progress to larger ulcers, accompanied by hemorrhagic necrosis and eschar-like changes; in some cases, the lesions may reach a diameter of up to… 4cm Pathologically, there is angiotropic infiltration by pleomorphic lymphocytes; in some patients, vascular destruction may be observed, and intravascular thrombi may also occur.
1. 5 F Type Namely, follicular-type Ly P. 1980 year Pierard Wait until the first report was filed. 2 In this case, papules and nodules occur intermittently at the hair follicles or around ruptured follicles. Histopathologically, there is infiltration of atypical lymphocytes and inflammatory cells in the superficial dermis surrounding the hair follicles, with multinucleated R cells occasionally observed. Eed-Sternberg Sample cell. Kempf Wait until it becomes follicular. Ly P The characteristics are summarized as : Within the hair follicle and around the hair follicle CD30 + Cellular infiltration, cyst wall distension, follicular rupture, proliferation of follicular epithelium, and neutrophil accumulation within the follicular epithelium. However, some studies have also reported findings across various types. Ly P The perifollicular phenomenon can be observed in all cases; therefore, it is perifollicular in nature. Ly P Whether it constitutes an independent subtype remains debatable.
1. 6 6p25. 3 Chromosomal rearrangement type 2013 Karai Wait until the first report was filed. 11 For example, histologically it manifests as a biphasic growth pattern. Ly P . This type of case is characterized by two distinct types of neoplastic cells: the superficial component consists of cerebriform small lymphocytes with epidermotropic behavior, which within the epidermis exhibit a pattern resembling Pagetoid reticulosis; the deep component comprises larger, anaplastic lymphocytes that proliferate in sheets and nodular patterns. The neoplastic cells express CD30 , in which the staining intensity of superficial small tumor cells is weaker than that of deep-seated large tumor cells. A distinctive molecular biological feature of such cases is chromosomal alterations in the tumor cells. 6p25. 3 on the DUSP22-I R F4 Gene rearrangement. Some scholars believe that 6p25. 3 The rearrangement leads to DUSP22 Its expression is reduced, thereby attenuating its tumor-suppressive effects and potentially contributing to tumorigenesis.
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Special type of Ly P
2. 1 Clinical Special Types In rare cases, Ly P may present with patchy changes, hemangioma‑like lesions, or findings resembling vaccinia‑like vesiculobullous disease. In some patients, the rash may occur at specific anatomical sites, such as the eyelids. Additionally, in a small number of cases, the rash is confined to a discrete area of the body; in certain individuals, it may be restricted to the region over a Becker nevus or arise on the background of a tattoo.
2. 2 Pathological special types 2003 Crowson Wait until the first report was filed. 9 Example: Apocrine type Ly P , and there is cellular infiltration centered on the sweat glands, manifesting in two patterns. : One pattern is characterized by a predominance of small lymphocytes, with sparse infiltration of larger cells. ; Another pattern is a polymorphic cellular infiltrate accompanied by granulomas. Ly P Reactive epidermal hyperplasia may occur; previous reports have described it as being associated with keratoacanthoma, and similar findings have also been documented in primary cutaneous anaplastic large cell lymphoma. This so‑called keratoacanthoma‑like change likely reflects reactive epidermal hyperplasia superimposed on the underlying lymphoma, rather than a true keratoacanthoma.
2. 3 Immunohistochemical special phenotype Ly P can be expressed CD56 Mark, but CD56 Whether or not it is expressed is unrelated to prognosis. Recent literature has reported that… Ly P γδ may appear. T Phenotype, and γδ T Phenotypic Ly P Nor is there any obvious invasive clinical behavior.
A small number of cases have been reported. CD30 Negative Ly P , including those reported recently CD30 Negative D Type Ly P 。 Cerroni to think that CD30 A negative result may be due to laboratory technical issues, but other researchers believe it is genuine. CD30 Negative cases.
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The relationship between Ly P and other malignant lymphomas
Approximately 10% to 20% of the Ly P The patient has an associated malignant lymphoma; in a recent large‑scale retrospective study, the incidence of such lymphomas was found to be as high as 52% . Some studies have found that TC R gene rearrangement positivity is associated with the development of related lymphomas, and the incidence of these lymphomas has been observed to follow a continuous pattern: it gradually increases over time, at… 18 Stability was only achieved after the New Year. Studies with longer follow-up periods reported higher incidence rates of the relevant lymphomas compared to those with shorter follow-up durations. According to previous literature, in… Ly P Among patients, the highest incidence is observed for mycosis fungoides and primary cutaneous anaplastic large-cell lymphoma, which are associated with… Ly P The genetic associations have also been confirmed by multiple studies.
4. Advances in the Treatment of Ly P
Ly P is an indolent disease with self‑healing characteristics; treatment is typically aimed at controlling the widespread rash and reducing the frequency of relapses. Currently, there is no evidence that any existing therapeutic regimen can decrease the frequency or number of relapses or prevent the development of associated lymphomas, and skin lesions usually recur upon discontinuation of treatment.
4.1 Topical Pharmacotherapy Topical corticosteroids are commonly used and may serve as first-line therapy for patients seeking rapid symptom relief; however, they fail to achieve complete remission in most cases. Recently, some researchers have reported favorable outcomes with topical methotrexate (MTX). Other topical agents that have been employed in treatment include… : Nitrogen mustard, imiquimod, vitamin A Acidic drugs, etc.
4. 2 Phototherapy Ultraviolet therapy can induce lymphocyte apoptosis, has relatively few side effects, and can also be used to treat LyP. Some researchers have attempted to use… PUVA 、 UVA1 and also 308 Nanosecond excimer laser therapy has been successful in most patients.
4.3 Systemic Treatment MTX is usually used as Ly P the first-line systemic therapy. In a study that included … 40 Example Ly P In the retrospective study of patients, all were administered MTX 15-25mg Subcutaneous injection 1 / Weekly treatment, 44% The patient has no new skin lesions, 42% Patients had only a small number of new skin lesions. After discontinuation of treatment, in… 24-227 During the one-month follow-up period 25% No patients experienced recurrence. However, most patients developed adverse effects of varying severity, among which… 10 a use MTX Treatment exceeding 3 Among the patients of the year, there are 5 Humans develop hepatic fibrosis. Studies have found that, IFN- α for Ly P It exhibits long-lasting, sustained effects, possibly due to its induction of high efficacy. January Type immune response and promotion of apoptosis receptors CD95 Expression and related aspects. In an open-label experiment, participants were administered weekly… 3-15MU IFN- α therapy, ongoing 2-6 A week later, 5 Among the patients, there are 4 For example, complete remission was achieved. After discontinuation of treatment, in… 3-4 Relapse may occur within the week; therefore, maintenance therapy is necessary to achieve… 10-17 months. The latest experimental data indicate that the combined use of MTX and IFN- Alpha therapy Ly P Effective. When using Brentuximab vedotin (CD30) Antibody-drug conjugates c AC10-vc MMAE) 1. 8 mg/kg21 Heaven / Clinical aspects of intravenous injection therapy 2 In the study, all of the Ly P All treatments are effective, with the primary adverse reaction being peripheral sensory neuropathy. Based on clinical practice, CD30 Monoclonal antibodies are expensive and carry certain adverse effects, so they remain some distance from widespread clinical application.
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Conclusion
LyP is a low-grade malignancy, and its diagnosis requires close integration of clinical and pathological findings. CD30 Staining has diagnostic value. Currently, in… Ly P In terms of research, several clinical issues remain that warrant further investigation, such as… Ly P With and PLEVA Is it a hereditary disease, specifically follicular? Ly P Whether it is a specific subtype, Ly P The relationship with mycosis fungoides and other types of lymphoma, among others.
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