RESUMO
DUSP22-rearranged primary cutaneous anaplastic large-cell lymphoma (pcALCL) has a biphasic histological pattern defined by large dermal atypical lymphocytes and epidermotropic small lymphocytes resembling pagetoid reticulosis, but the positivity rate of the biphasic pattern in DUSP22-rearranged pcALCL is unknown. Immunohistochemically, LEF1 expression in >75% of tumor cells is associated with DUSP22-rearrangement (DUSP22-R) in systemic ALCL. However, whether this association applies to pcALCL remains unclear. To analyze these pathological clues for screening DUSP22-R, we reviewed 11 skin biopsies from three patients with DUSP22-rearranged pcALCL. All specimens showed a biphasic pattern, of which three showed nonpagetoid infiltration of the epidermis. In all lesions, small-cell changes of tumor cells were observed not only within the epidermis but also under the epidermis. LEF1 positivity rates varied by lesion (range: 30%-90%, mean: 59.6%) with only three patients expressing LEF1 in more than 75% of tumor cells. In conclusion, the biphasic pattern was a constant finding in DUSP22-rearranged pcALCL, but it was not always pagetoid reticulosis-like. The recognition of small-cell change outside the epidermis may be helpful in diagnosing DUSP22-rearranged pcALCL. However, LEF1 expression was variable and its diagnostic usefulness may be limited.
Assuntos
Linfoma Anaplásico de Células Grandes , Reticulose Pagetoide , Neoplasias Cutâneas , Humanos , Linfoma Anaplásico de Células Grandes/patologia , Biópsia , Neoplasias Cutâneas/patologia , Fator 1 de Ligação ao Facilitador Linfoide/genética , Fosfatases de Especificidade Dupla/genética , Fosfatases da Proteína Quinase Ativada por Mitógeno/genéticaRESUMO
OBJECTIVE: Woringer-Kolopp disease (WKD), also known as localized pagetoid reticulosis, is a rare variant of mycosis fungoides as described by the World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC) classification system for cutaneous lymphomas. Our objective was to conduct a comprehensive review that describes and evaluates patient demographics, clinical presentation, immunohistochemical findings, management, and outcomes of WKD. METHODS: The databases PubMed, Embase, and Cochrane Library were searched for relevant literature. Patient demographics, imaging, treatments, and other clinical characteristics were obtained. The literature search was conducted on December 7, 2020. Studies were included if they contained primary data related to WKD. Non-pertinent studies, non-English studies, non-human studies, review articles, or studies with insufficient case information were excluded. The quality of the included studies and the risk of bias were evaluated using the Grades of Recommendation, Assessment, Development, and Evaluation Group (GRADE) criteria (2016), Oxford Centre for Evidence-Based Medicine: Levels of Evidence (OCEBM) (March 2009), and the Methodological Index for Non-Randomized Studies (MINORS) criteria (2003). RESULTS: A total of 84 studies with 143 patients met the inclusion criteria for this study. The most common chief complaint was an asymptomatic, slow-growing, and erythematous plaque. The average time from initial skin eruption to presentation was 97.6 months. Histologically, 128 cases of WKD displayed epidermotropism (97.7%). Various therapies, including radiotherapy, surgery, and local chemotherapy, were used. In total, 92 (78.6%) cases had complete remission and 11 (9.4%) cases recurred. CONCLUSIONS: WKD is a rare cutaneous T-cell lymphoma that often presents as a single plaque on the extremities with an indolent course and a favorable prognosis. However, it is often misdiagnosed, leading to delays in treatment. The diagnosis of WKD involves clinical appraisal, a biopsy of suspicious lesions, and immunohistochemistry. Monotherapy appears to be preferred compared to combination therapy for WKD with radiotherapy demonstrating among the highest cure rates and lowest recurrence. Future retrospective and prospective studies are needed to accurately determine the epidemiology, prognosis, and effective treatments for WKD. Limitations include a possibility of missed studies, heterogeneity in reporting methods, publication, and selection bias.
Assuntos
Linfoma Cutâneo de Células T , Micose Fungoide , Reticulose Pagetoide , Neoplasias Cutâneas , Humanos , Reticulose Pagetoide/patologia , Estudos Retrospectivos , Micose Fungoide/patologia , Neoplasias Cutâneas/patologiaAssuntos
Reticulose Pagetoide , Neoplasias Cutâneas , Criança , Humanos , Masculino , População do Leste AsiáticoRESUMO
A 70-year-old male presented with reddish brown, keratotic plaque on his left leg which had grown slowly for ten years. The histopathological examination revealed acanthosis and bands of lymphocytes with significant epidermotropism. The intraepidermal lymphocytes showed large, pleomorphic and hyperchromatic nuclei with distinct nucleoli and perinuclear halos, positive for CD3, CD4 and CD30, negative for CD8, CD20 and high in Ki67 index. Accordingly, the diagnosis of Pagetoid reticulosis was made. The patient was then treated with topical photodynamic therapy once a week sequential to partial in-situ resection and preliminary skin dermabrasion and finally achieved complete remission without obvious scars or recurrence after four sessions.
Assuntos
Reticulose Pagetoide , Fotoquimioterapia , Neoplasias Cutâneas , Idoso , Humanos , Masculino , Reticulose Pagetoide/tratamento farmacológico , Reticulose Pagetoide/patologia , Fotoquimioterapia/métodos , Pele/patologia , Neoplasias Cutâneas/patologiaAssuntos
Leucemia-Linfoma de Células T do Adulto , Reticulose Pagetoide , Dermatopatias , Neoplasias Cutâneas , Humanos , Leucemia-Linfoma de Células T do Adulto/complicações , Leucemia-Linfoma de Células T do Adulto/diagnóstico , Leucemia-Linfoma de Células T do Adulto/tratamento farmacológico , Pele , Neoplasias Cutâneas/diagnósticoRESUMO
Woringer-Kolopp disease is a rare variant of mycosis fungoides, a type of cutaneous T-cell lymphoma. Described is a case of a small annular plaque on the foot diagnosed histologically as Woringer-Kolopp disease and treated successfully with topical and intralesional steroids. In addition, a brief review of the literature and treatment options is provided.
Assuntos
Micose Fungoide , Reticulose Pagetoide , Neoplasias Cutâneas , HumanosRESUMO
Pseudocarcinomatous hyperplasia (PCH) is a benign reactive epithelial proliferation that may be associated to lymphomas of the skin or external mucous membranes. We present a case of single lesion mycosis fungoides (Woringer-Kollop's reticulosis pagetoid) associated with PCH that was initially misdiagnosed as squamous cell carcinoma (SCC) and review all PubMed-indexed previously reported cases on lymphomas of the skin or external mucous membranes associated to PCH, SCC, and keratoacanthomas. Including our own case, we collected data of 114 cases of cutaneous or mucosal lymphoproliferative disorders associated to PCH, 3 cases associated to SCC, and other 3 cases associated to keratoacanthomas. All cases were tabulated to the following parameters whenever data was available: sex, age, previous medical conditions, number of lesions (single × multiple), site of involvement (mucosa, skin or both), clinical impression, initial equivocal histopathologic diagnosis, final diagnosis, keratinocytic atypia (presence × absence), lymphocytic atypia (presence × absence), CD30-status, and treatment.
Assuntos
Carcinoma de Células Escamosas/patologia , Ceratoacantoma/patologia , Mucosa/patologia , Reticulose Pagetoide/patologia , Neoplasias Cutâneas/patologia , Pele/patologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Biomarcadores Tumorais/análise , Biópsia , Carcinoma de Células Escamosas/química , Carcinoma de Células Escamosas/terapia , Criança , Erros de Diagnóstico , Feminino , Humanos , Hiperplasia , Imuno-Histoquímica , Ceratoacantoma/metabolismo , Ceratoacantoma/terapia , Masculino , Pessoa de Meia-Idade , Mucosa/química , Reticulose Pagetoide/química , Reticulose Pagetoide/terapia , Valor Preditivo dos Testes , Pele/química , Neoplasias Cutâneas/química , Neoplasias Cutâneas/terapia , Adulto JovemRESUMO
Pagetoid reticulosis (PR) is a rare lymphoproliferative disorder with indolent behavior considered a variant of mycosis fungoides. It is characterized by marked epidermotropism of the neoplastic lymphocytes. Since its original description, five cases have been reported in children. We report a new case of PR with an immunohistochemical profile not previously described in children.
Assuntos
Imuno-Histoquímica/métodos , Reticulose Pagetoide/metabolismo , Neoplasias Cutâneas/patologia , Administração Tópica , Adolescente , Biópsia , Criança , Pré-Escolar , Clobetasol/administração & dosagem , Clobetasol/uso terapêutico , Feminino , Glucocorticoides/administração & dosagem , Glucocorticoides/uso terapêutico , Humanos , Imunofenotipagem/métodos , Linfócitos/patologia , Linfócitos/ultraestrutura , Transtornos Linfoproliferativos/patologia , Masculino , Micose Fungoide/patologia , Pele/patologia , Resultado do TratamentoRESUMO
Reticulose pagetoide é a expressão utilizada para uma das mais raras formas de linfoma descritas em cães. Trata-se de um distúrbio linfoproliferativo cutâneo que emerge de linfócitos T intraepidérmicos. Descreve-se aqui um caso de reticulose pagetoide com apresentação localizada, muito semelhante à doença de Woringer-Kolopp dos humanos, que acometeu um cão, Boxer, fêmea de 10 anos de idade. O paciente foi atendido devido ao aparecimento de um nódulo na região nasolabial, com evolução clínica de cerca de 30 dias. Histologicamente o nódulo era composto por uma proliferação linfoide intraepidérmica e anexal. Os linfócitos neoplásicos, células T confirmadas por meio da imunomarcação positiva para CD3 e negativa para CD79, eram grandes células redondas e monomórficas. Apesar de rara, a reticulose pagetoide é um distúrbio linfoproliferativo de fácil suspeita com base apenas na morfologia celular. Esse neoplasma deverá ser lembrado sempre que um padrão linfoide intraepidérmico estiver presente.(AU)
Reticulosis pagetoide is an expression used for one of the rare forms of lymphoma described in dogs. It is a cutaneous lymphoproliferative disorder that emerges from intraepidermal T-cells. We describe a case of pagetoid reticulosis with localized presentation, very similar to Woringer-Kolopp's disease in humans, which affected a 10-year-old Boxer female. The patient was seen due to the appearance of a nodule in the nasolabial region with clinical evolution of about 30 days. Histologically, it was composed of an intraepidermal and annexal lymphoid proliferation. Neoplastic lymphocytes, T-cells confirmed by CD3-positive and CD79-negative immunolabeling, were large, round, and monomorphic cells. Although rare, pagetoid reticulosis is an easily suspected lymphoproliferative disorder based only on cell morphology. This neoplasm should be remembered whenever an intraepidermal lymphoid pattern is present.(AU)
Assuntos
Animais , Feminino , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/veterinária , Doenças do Cão , Reticulose Pagetoide/diagnóstico , Reticulose Pagetoide/veterináriaRESUMO
Pagetoid reticulosis (PR), also known as Woringer-Kolopp disease, is a rare variant of mycosis fungoides with distinctive clinicopathologic features. It clinically manifests as a solitary, erythematous, gradually enlarging, scaly, or verrucous plaque on the lower extremities, and due to its indolent course and nonspecific clinical features, may remain undiagnosed for years. In the current study, we describe the clinical and dermoscopic characteristics of a rare case of PR disease and correlate them with the corresponding histopathologic findings. Dermoscopy may prove beneficial in early diagnosis of this rare entity.
Assuntos
Dermoscopia , Reticulose Pagetoide , Neoplasias Cutâneas , Pele/patologia , Idoso , Humanos , Masculino , Reticulose Pagetoide/diagnóstico por imagem , Reticulose Pagetoide/patologia , Neoplasias Cutâneas/diagnóstico por imagem , Neoplasias Cutâneas/patologiaRESUMO
Pagetoid reticulosis is an indolent cutaneous T-cell lymphoma and presents as erythema or plaque with a well-defined border on the distal areas of the extremities. Immunophenotypic studies show that in most cases, neoplastic lymphocytes are positive for CD4, whereas CD20 and CD30 double positivity was rarely reported. In this paper, we report an 80-year-old woman who presented with erythema on the extremities for 3 years. Skin biopsy on the right forearm was performed. Histopathologically, the erythematous lesions were characterized by atypical lymphocytes with significant epidermotropism. Immunohistochemical staining showed high proliferation as evidenced by high Ki-67 index and that the tumor cells were positive for CD20 and CD30 but negative for CD7 and CD56. The patient was treated with one cycle of radiotherapy and is currently doing well.
Assuntos
Antígenos CD20/genética , Antígeno Ki-1/genética , Reticulose Pagetoide/diagnóstico , Neoplasias Cutâneas/diagnóstico , Idoso de 80 Anos ou mais , Biópsia , Eritema , Feminino , Humanos , Imunofenotipagem , Linfócitos , Linfoma Cutâneo de Células T/diagnóstico , Reticulose Pagetoide/radioterapia , Pele/patologia , Neoplasias Cutâneas/radioterapia , Resultado do TratamentoAssuntos
Reticulose Pagetoide/patologia , Neoplasias Cutâneas/patologia , Adulto , Pé/patologia , Humanos , MasculinoAssuntos
Dermatoses do Pé/diagnóstico , Reticulose Pagetoide/diagnóstico , Neoplasias Cutâneas/diagnóstico , Idoso de 80 Anos ou mais , Carcinoma de Células Escamosas , Diagnóstico Diferencial , Dermatoses do Pé/patologia , Humanos , Masculino , Melanoma , Reticulose Pagetoide/patologia , Neoplasias Cutâneas/patologiaRESUMO
A 9-year-old, intact female alpaca (Vicugna pacos) was presented for a second opinion with a 1-year history of nonpruritic, multifocal scaling and crusted cutaneous lesions, mainly involving skin on the face, axillae, and ventral abdomen. Clinical abnormalities were limited to the skin, and the alpaca was otherwise healthy. The initial veterinarian had examined the alpaca, found no evidence of ectoparasites with laboratory testing, and had tried several trial therapies including oral antibiotics, ivermectin, and topical use of betadine solution. At the time of presentation, the lesions had neither improved nor worsened with any attempted therapy, and multiple skin biopsies were collected. Histopathology and immunohistochemical staining findings were consistent with the pagetoid reticulosis type of cutaneous epitheliotropic T-cell lymphoma. Our report describes the clinical, histopathologic, and immunophenotypic features of pagetoid reticulosis epitheliotropic cutaneous T-cell lymphoma in an alpaca.
Assuntos
Camelídeos Americanos , Reticulose Pagetoide/veterinária , Neoplasias Cutâneas/veterinária , Animais , Feminino , Imunofenotipagem/veterinária , Reticulose Pagetoide/diagnóstico , Reticulose Pagetoide/etiologia , Reticulose Pagetoide/patologia , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/etiologia , Neoplasias Cutâneas/patologiaRESUMO
Woringer-Kolopp (WK) is a rare subtype of cutaneous T-cell lymphoma (CTCL) with limited treatment options. Bexarotene gel is a topical retinoid used in the treatment of CTCL. This report describes three female patients (mean age 66 years) with WK disease who had an effective treatment response to bexarotene 1% gel. This treatment could provide a safe alternative to other current treatment modalities which have higher risks of potential adverse effects and lack of access to other conventional treatments such as light therapy.