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Abstract Background An increased risk of Secondary Malignancies (SMs) in Mycosis Fungoides (MF) has been suggested previously. However, the relationship between this risk and the features of MF is not well-known. Objective To investigate the rate and types of SMs in a large cohort of MF patients focusing on the associated features of these patients. Methods The demographic features, subtype, and stage of MF, as well as the temporal relationship between the diagnosis of MF and the development of SMs were determined. Major clinical features of MF in this group were compared with MF patients without association of SMs. Results Among 730 MF patients with a mean follow-up period of 67.9 ± 52.4 months, 56 SMs were identified in a total of 52 (7.1%) patients. While 28.8% of patients were previously diagnosed with other malignancies, then subsequently had a diagnosis of MF, it was vice versa in 53.8% of patients. Most of the SM-associated MF patients had early-stage (80.7%) and classical type of MF (86.5%) without a significant difference from MF patients without association of SMs; 85.5% and 72.5%, respectively. The most commonly identified SMs were hematologic malignancies (64.3%) including lymphomatoid papulosis (n = 22), Hodgkin's lymphoma (n = 4), non-Hodgkin's lymphoma (n = 5), polycythemia vera (n = 2). Other most commonly associated malignancies were breast cancer (n = 4), prostate cancer (n = 3), renal cell carcinoma (n = 2), melanoma (n = 2), and Kaposi's sarcoma (n = 2). Study limitations A single tertiary dermatology center study with a retrospective design. Conclusion Apart from the well-known lymphomatoid papulosis association, systemic hematological malignancies were also quite common in the large cohort of MF patients.
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Objective:To analyze and summarize clinical and pathological characteristics of clear cell papulosis (CCP) .Methods:Clinical data were collected from 57 children with CCP, who were diagnosed in Department of Dermatology, Children′s Hospital of Chongqing Medical University from November 2014 to March 2022, and their clinical and pathological characteristics were analyzed.Results:Among the 57 patients, 30 were males and 27 were females; their age ranged from 6 months to 7 years and 10 months; the age at onset ranged from 1 to 74 months, and 39 (68.4%) experienced onset of disease in infancy; the course of disease ranged from 1 to 84 months. Skin lesions mainly manifested as millet- to soybean-sized roundish white macules or short bar-like white macules measuring 1 - 2 cm in length. Most patients (49 cases, 85.9%) had skin lesions less than 20 in number. The most common location of skin lesions was the lower abdomen (35 cases, 61.4%), followed in turn by chest or upper abdomen (34 cases, 59.6%), anterior axilla or armpit (17 cases, 29.8%), and vulva (11 cases, 19.3%). Short bar-like white macules predominated on the chest or upper abdomen (23/34, 65.7%) and anterior axilla or armpit (10/17), roundish white macules predominated on the lower abdomen (30/35, 85.7%), and the vulvar lesions all manifested as roundish white macules. Histopathological findings showed that slightly larger cells with abundant clear cytoplasm were scattered in or above the basal layer of the epidermis, and these cells were positive for cytokeratin 7, carcinoembryonic antigen, and periodic acid-Schiff staining.Conclusion:CCP mainly occurred in young children, roundish white macules predominated on the lower abdomen and vulva, and short bar-like white macules predominated on the chest or upper abdomen, as well as on the anterior axilla or armpit, indicating that CCP has strongly identifiable clinical features.
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Fibroelastolytic papulosis is an acquired elastic tissue disorder that includes the clinicopathological features of white fibrous papulosis of the neck (WFPN) and pseudoxanthoma elasticum-like papillary dermal elastosis (PXE-PDE). It is clinically characterized by discrete white papules occurring on the neck in middle-aged women and is histopathologically characterized by elastolysis and/or fibrosis in the upper dermis. An 80-year-old woman presented with multiple white papules on her anterior chest, which occurred 6 months prior to the presentation. Histopathological examination of a biopsy specimen revealed some degree of fibrosis and elastolysis in the upper dermis. Thus, the final diagnosis in this case was compatible with fibroelastolytic papulosis considering that this patient showed clinicopathological features of both WFPN and PXE-PDE. Notably, this is the first case report that describes fibroelastolytic papulosis confined exclusively to the anterior chest.
Subject(s)
Aged, 80 and over , Female , Humans , Biopsy , Dermis , Diagnosis , Elastic Tissue , Fibrosis , Neck , ThoraxABSTRACT
Lymphomatoid papulosis type E (LyP) is a recently described subtype of LyP characterized by an angioinvasive infiltrate of atypical lymphocytes expressing CD30. We present a case of type E LyP with extensive cutaneous necrosis in the histopathological evaluation which was misdiagnosed as an ulcerative form of bacterial skin infection. The remarkable cutaneous necrosis showed in our case might be related to the angiodestructive infiltrate that was present in this circumstance.
ABSTRACT
Lymphomatoid papulosis type E (LyP) is a recently described subtype of LyP characterized by an angioinvasive infiltrate of atypical lymphocytes expressing CD30. We present a case of type E LyP with extensive cutaneous necrosis in the histopathological evaluation which was misdiagnosed as an ulcerative form of bacterial skin infection. The remarkable cutaneous necrosis showed in our case might be related to the angiodestructive infiltrate that was present in this circumstance.
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La papulosis fibrosa blanca del cuello es una patología benigna, que se presenta frecuentemente en personas mayores y que se caracteriza por pápulas blanquecinas ubicadas habitualmente en las regiones laterales del cuello. El hallazgo histopatológico distintivo es el incremento de las fibras de colágeno en la dermis papilar. Su etiopatogenia es, hasta ahora, desconocida y su tratamiento es difícil. Dado que corresponde a una patología infrecuente, probablemente subdiagnosticada, es que se decide reportar el caso de una mujer chilena de 77 años.
White fibrous papulosis of the neck is a benign pathology, which usually occurs in old people and is characterized by whitish papules that are frequently located in the lateral regions of the neck. The distinctive histopathological finding is the increase of collagen fibers in the papillary dermis. Its pathophysiology is not currently known and its treatment is difficult. Due to being a rare pathology, probably underdiagnosed, it was decided to report the case of a 77-yearold chilean woman.
Subject(s)
Humans , Female , Aged , Skin Aging/pathology , Skin Diseases, Papulosquamous/pathology , Neck/pathology , Biopsy , Diagnosis, DifferentialABSTRACT
La papulosis linfomatoide forma parte del espectro de los procesos linfoproliferativos cutáneos primarios de células T CD30+. Es una enfermedad rara de etiopatogenia incierta y compleja. El diagnóstico diferencial puede a veces resultar muy difícil. Se describió el caso de una mujer de 80 años con el diagnóstico, particularmente atípico desde la visión histopatológica, en el cual la correlación anatomoclínica ha sido un importante aspecto que lo hace interesante. El objetivo es comunicar un caso de presentación poco frecuente en la práctica médica (AU).
Lymphomatoid papulosis is part of the primary skin lymph proliferative processes of the T CD30+ cells. It is a rare disease of complex and uncertain etiopathogenesis. The differential diagnosis could be very difficult sometimes. The described case was the one of a female patient, aged 80 years with that diagnosis, particularly atypical from the histopathological point of view, where the anatomoclinical correlation has been an important aspect making it interesting. The objective is to inform a case of infrequent presentation in the medical practice (AU).
Subject(s)
Humans , Female , Adult , Skin Neoplasms/epidemiology , Lymphomatoid Papulosis/epidemiology , Medical Records , Lymphomatoid Papulosis/complications , Lymphomatoid Papulosis/diagnosis , Lymphomatoid Papulosis/pathology , Diagnosis, Differential , Degloving Injuries/diagnosis , Lymphoma/diagnosisABSTRACT
Lymphomatoid papulosis (LyP) is defined as a chronic, recurrent, self-healing papulonecrotic or papulonodular skin disease with histologic features suggestive of a (CD30-positive) malignant lymphoma. In up to 20% of patients, LyP are preceded by, associated with, or followed by another type of cutaneous or systemic lymphoma, generally mycosis fungoides (MF), primary cutaneous anaplastic large cell lymphoma (C-ALCL). In this case, we describe a case of MF that preceded and continued to coexist with LyP type C.(AU)
A papulose linfomatóide (LyP) é definida como uma doença cutânea papulonecrótica ou papulonodular crônica, recorrente, com características histológicas sugestivas de linfoma maligno (CD30-positivo). Em até 20% dos pacientes, o LyP é precedido por, associado ou seguido por outro tipo de linfoma cutâneo ou sistêmico, geralmente micose fungóide (MF), linfoma cutâneo primário de células grandes anaplásicas (C-ALCL). Neste caso, descrevemos um caso de MF que precedeu e continuou a coexistir com LyP tipo C. (AU)
Subject(s)
Humans , Female , Adult , Lymphoma , Lymphoma, Primary Cutaneous Anaplastic Large Cell , Lymphomatoid Papulosis , Mycosis Fungoides , T-LymphocytesABSTRACT
Abstract: The classification of cutaneous lymphomas is multidisciplinary and requires the correlation between clinical, histopathological, immunohistochemical, and molecular diagnostic elements. In this article, we present four different cases of CD30-positive T-cell lymphoma with cutaneous manifestations. We compare cases with definitive diagnosis of papulosis lymphomatoid type C, primary cutaneous anaplastic large T-cell lymphoma, systemic anaplastic large T-cell lymphoma with secondary skin involvement, and mycosis fungoides with large cell transformation, highlighting the importance of clinicopathological correlation to classify these cases.
Subject(s)
Humans , Male , Female , Adolescent , Middle Aged , Aged, 80 and over , Skin Neoplasms/pathology , Lymphoma, Primary Cutaneous Anaplastic Large Cell/pathology , ImmunohistochemistryABSTRACT
Objective@#To study the clinicopathologic characteristics and immunophenotype of lymphomatoid papulosis(LyP), followed by exon mutation analysis with focus on gene mutations involved in apoptosis pathway and other possible pathogenic genes.@*Methods@#Clinical data analysis and immunohistochemical staining were carried out in 20 cases of LyP. Whole exome sequencing technology was employed in 2 cases of type C of LyP.@*Results@#Of the 20 cases, there were 9 males and 11 females with a median age of 28.6 years. Nineteen patients presented with multiple papules and nodules, and one case presented with only one tumor nodule. Of the fifteen cases with available followed-up data, all were alive (20-155 months). Histologically, the tumors primarily involved the dermis and subcutaneous layer, in which 6 were type A, 3 were type B, 10 were type C and 1 was type D. Main infiltration patterns included wedge-shaped, band-like, sheets and large nodular. Immunohistochemistry showed that most cases expressed CD30 in the large tumor cells. Sixteen cases expressed CD3, 17 cases expressed CD4 and 8 cases expressed CD8. Sixteen cases expressed TIA1. Ten cases expressed GrB and 1 case expressed CD15. All but one case did not expressed CD20. All cases did not express ALK1.A total of 101 common non-synonymous mutations were detected in 2 cases of LyP type C by whole exome sequencing, including 87 missense mutations, 6 missense mutation/frame-shift deletions, 2 missense mutation/nonframe-shift deletions, 5 frame-shift deletions, 1 missense mutations/synonymous mutation. Syndecan-1(SDC1), COL4A1, Laminin-5 were involved in the extracellular matrix receptor pathway.@*Conclusions@#Clinical presentations are crucial for the diagnosis of LyP. LyP has a favorable prognosis. SDC1, COL4A1 and Laminin-5 gene mutations may be associated with tumor recurrence or progression into a higher gradelymphoma.
ABSTRACT
Clear cell papulosis (CCP) is a rare skin condition characterized by asymptomatic multiple hypopigmented maculopapules distributed primarily on the lower aspect of the abdomen or along the milk lines in young children. The characteristic histological feature of CCP is the presence of clear cells scattered among basal keratinocytes. We describe the case of a 3-year-old girl with clear cell papulosis. Immunohistochemical staining results were variable based on the different kinds of CEA antibodies. Immunostaining specification of CEA glycoproteins may provide a clue for the differential diagnosis of clear cell papulosis with other diseases such as primary extramammary Paget's disease.
Subject(s)
Child , Child, Preschool , Female , Humans , Abdomen , Antibodies , Carcinoembryonic Antigen , Diagnosis, Differential , Glycoproteins , Immunohistochemistry , Keratinocytes , Milk , Paget Disease, Extramammary , SkinABSTRACT
Clear cell papulosis (CCP) is a rare skin condition characterized by asymptomatic multiple hypopigmented maculopapules distributed primarily on the lower aspect of the abdomen or along the milk lines in young children. The characteristic histological feature of CCP is the presence of clear cells scattered among basal keratinocytes. We describe the case of a 3-year-old girl with clear cell papulosis. Immunohistochemical staining results were variable based on the different kinds of CEA antibodies. Immunostaining specification of CEA glycoproteins may provide a clue for the differential diagnosis of clear cell papulosis with other diseases such as primary extramammary Paget's disease.
Subject(s)
Child , Child, Preschool , Female , Humans , Abdomen , Antibodies , Carcinoembryonic Antigen , Diagnosis, Differential , Glycoproteins , Immunohistochemistry , Keratinocytes , Milk , Paget Disease, Extramammary , SkinABSTRACT
La papulosis bowenoide es un carcinoma espinocelular in situ de bajo grado que se observa mayormente en genitales. El agente etiológico es el virus del papiloma humano. Existen múltiples opciones terapéuticas médicas y quirúrgicas pero debería ser tratado de forma conservadora. Presentamos dos casos de papulosis bowenoide tratados exitosamente con crioterapia (AU)
Bowenoid papulosis is a low grade squamous cell in situ carcinoma. It is usually seen in genitals. Etiologic agent is the human papilloma virus. There are many treatments available but it should be treated conservatively. Two cases of bowenoid papulosis with good response to cryotherapy are reported (AU)
Subject(s)
Humans , Male , Adult , Papillomaviridae/pathogenicity , Penile Neoplasms/etiology , Carcinoma in Situ/diagnosis , Therapeutics , CryotherapyABSTRACT
Three histological subtypes of lymphomatoid papulosis (LyP), type A (histiocytic), type B (mycosis fungoides like) and type C (anaplastic large cell lymphoma like) are well recognized. Two new histological variants, type D (simulating an aggressive epidermotropic cytotoxic lymphoma) and type E (angioinvasive type) has been described recently. We describe a 27‑year‑old man presented with a history of asymptomatic erythematous papules on both upper and lower limbs noted since 10 years of age. There were no systemic symptoms. Biopsy revealed an atypical dermal lymphoid infiltrate with epidermotropism, and the immunohistochemical markers showed a diffuse positivity for CD3, CD8, CD56, T1A and granzyme B with the focal positivity of CD30. All other relevant tests were normal. In this case report of a recently described delineated variant of LyP we emphasize the indolent course of this entity although the histology would suggest a more aggressive disease.