ABSTRACT
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
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.
ABSTRACT
Comunicamos el caso de una papulosis linfomatoide en una mujer de 38 años, el tipo histológico es B. Efectuamos una revisión de la entidad y sus interrogantes aún no resueltos. Se reivindica a su descubridor y el nombre inicial de su enfermedad: Warren L Macaulay y erupción rítmica paradojal, respectivamente.
A 38 years-old woman with a type B of lymphomatoid papulosis, is reported. A review of this peculiar disease and its unresolved questions is made. We make tribute to the dermatologist who described the disease and the initial name: Warren L. Macaulay and rhythmic paradoxical eruption, respectively.
ABSTRACT
Comunicamos el caso de una papulosis linfomatoide en una mujer de 38 años, el tipo histológico es B. Efectuamos una revisión de la entidad y sus interrogantes aún no resueltos. Se reivindica a su descubridor y el nombre inicial de su enfermedad: Warren L Macaulay y erupción rítmica paradojal, respectivamente.
A 38 years-old woman with a type B of lymphomatoid papulosis, is reported. A review of this peculiar disease and its unresolved questions is made. We make tribute to the dermatologist who described the disease and the initial name: Warren L. Macaulay and rhythmic paradoxical eruption, respectively.
ABSTRACT
Patients with lymphomatoid papulosis have an increased risk (approx. 5% to 20%) of developing a malignant lymphoma such as mycosis fungoides, anaplastic large cell lymphoma (ALCL) and Hodgkin's disease before, during, or after lymphomatoid papulosis occurs. However, it is very rare that lymphomatoid papulosis occurs after ALCL, especially in childhood. An 11-year-old boy who had been diagnosed with ALCL 3 years prior and treated with chemotherapy and peripheral blood stem cell transplantation developed multiple scaly papules on his trunk and both extremities. Histopathologic and immunohistochemical examination of the scaly papules revealed lymphomatoid papulosis. The patient was cured with narrow band UVB treatment and there has been no relapse in lesions 10 years later. We report a case of lymphomatoid papulosis following allogenic stem cell transplantation for ALCL.
Subject(s)
Child , Humans , Male , Drug Therapy , Extremities , Hodgkin Disease , Lymphoma , Lymphoma, Large-Cell, Anaplastic , Lymphomatoid Papulosis , Mycosis Fungoides , Peripheral Blood Stem Cell Transplantation , Recurrence , Stem Cell TransplantationABSTRACT
Patients with lymphomatoid papulosis have other lymphomas in 10~20% of cases, most commonly mycosis fungoides, Hodgkin's disease, and anaplastic large cell lymphoma. In a series involving at least 40 patients with lymphomatoid papulosis, the association of lymphomatoid papulosis with mycosis fungoides ranged from approximately 7% to 18%. It is most important to distinguish lymphomatoid papulosis from CD30-positive large cell transformation of mycosis fungoides. Both conditions can be distinguished on clinical grounds, and clinical course is often the only distinguishing feature. We report a case of lymphomatoid papulosis developing in an mycosis fungoides lesion in a patient who received 3 rounds of narrow band UVB phototherapy and topical corticosteroid application.
Subject(s)
Humans , Hodgkin Disease , Lymphoma , Lymphoma, Large-Cell, Anaplastic , Lymphomatoid Papulosis , Mycosis Fungoides , PhototherapyABSTRACT
PURPOSE: Lymphomatoid papulosis (LyP) is one of the primary cutaneous CD30-positive lymphoproliferative disorders. LyP of the eyelid has rarely been reported. Herein, a case of typical LyP of the medial canthal area is reported. In addition, a literature review was performed. CASE SUMMARY: A 40-year-old female presented with a skin mass in the medial canthal area of the left eye that developed 2 months earlier. Initially, a focal skin lesion developed, and even with conservative treatment at a local clinic, progressed to a mass lesion having a central ulceration and adjacent edema. After 6 weeks, the adjacent edema had gradually decreased. On ophthalmic examination, the left medial canthal lesion was a 6 x 6 mm sized elevated mass with a central crater covered by crust. The clinical impression was keratoacanthoma. The lesion was widely excised and reconstructed by a full-thickness skin graft after an incisional biopsy. Histopathologic findings showed dermal infiltration of various inflammatory cells with atypical lymphocytes showing positivity to the CD30 antigen, and LyP was diagnosed. Systemic evaluation showed no evidence of systemic lymphoma and the patient has remained free of recurrence or systemic disease after a 1-year follow-up.
Subject(s)
Female , Humans , Ki-1 Antigen , Biopsy , Edema , Eye , Eyelids , Follow-Up Studies , Keratoacanthoma , Lymphocytes , Lymphoma , Lymphomatoid Papulosis , Lymphoproliferative Disorders , Recurrence , Skin , Transplants , UlcerABSTRACT
Lymphomatoid papulosis has been classically described as a chronic, recurrent and self-healing papulonecrotic or papulonodular skin eruption, which is clinically benign and histopathologically malignant. The histologic characteristics of lymphomatoid papulosis are suggestive of a cluster of differentiation 30+ (CD30+) malignant lymphoma, and it is best regarded as a low grade cutaneous T cell lymphoma (CTCL). We hereby report a case of granulomatous and eccrinotropic lymphomatoid papulosis in a 40- year-old male. There was no systemic involvement. The patient was treated with low dose oral methotrexate with good response.
ABSTRACT
Objective To analyze the clinicopathologic features of primary cutaneous CD30 positive lymphoproliferative disorders. Methods A clinical, pathological and immunohistochemical analysis was carried out in 4 cases of lymphomatoid papulosis and 5 cases of primary cutaneous anaplastic large cell lymphoma. Results Lymphomatoid papulosis was divided into 3 subtypes, A, B and C. The lymphomatoid papulosis of subtype A was pathologically characterized by pleomorphic anaplastic large cells or Steinberg-reed cells scattered or patchly distributed among many inflammatory cells; subtype B showed pathological changes characteristic of granuloma fungoides, and manifested as a broad infiltration zone of lymphocytes in dermis with scattered small- to middle-sized atypical gyrus-like lymphocytes; subtype C was characterized by a diffuse distribution of anaplastic large cells and could clinically subside spontaneously. Primary cutaneous anaplastic large cell lymphoma clinically manifested as subcutaneous nodules or papules, and was pathologically characterized by large, pleomorphic, round or ellipse cells with plentiful, eosinophilic or bicolor cytoplasm, large nuclei and obvious nucleoli. The neoplastic cells characteristically expressed CD30 antigen in both lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma, and all the cases showed a favorable prognosis.Conclusions Primary cutaneous CD30 positive lymphoproliferative disorders are a spectrum of cutaneous T cell lymphoma with favorable prognosis, and a synthetic analysis of clinical, histopathological and immunohistochemical findings is beneficial to the diagnosis of these entities.
ABSTRACT
Lymphomatoid papulosis (LyP) is defined as a histologically malignant, but clinically benign condition. It can appear as erythematous pink to purple papules or nodules. Immunophenotyping studies of the lymphomatoid papulosis lesions have shown a predominance of a CD4 expression and negativity for CD8. However, a positive CD8 expression has rarely been reported for LyP. Herein we report on a case of CD8 positive lymphomatoid papulosis in a 43-year-old man. The patient presented with erythematous, asymptomatic papules on the left axilla and thigh. Histopathologically, there was a wedge-shaped infiltrate composed of a mixture of various cell types, including lymphocytes, histiocytes, neutrophils and large atypical lymphoid cells. Immunophenotyping revealed the neoplastic cells were positive for CD3, CD8 and CD30 and they were negative for CD4, CD20 and CD56.
Subject(s)
Adult , Humans , Axilla , Histiocytes , Immunophenotyping , Lymphocytes , Lymphomatoid Papulosis , Neutrophils , ThighABSTRACT
Lymphomatoid papulosis is a lymphoproliferative disorder which is characterized by chronic recurrent self-healing papules or nodules with histologic features suggestive of a malignant lymphoma. An association of lymphomatoid papulosis with other lymphomas including mycosis fungoides, Hodgkin's disease and anaplastic large cell lymphoma is present in approximately 10 to 20% of cases. We herein report a case of lymphomatoid papulosis developed after remission of Hodgkin's disease.
Subject(s)
Hodgkin Disease , Lymphoma , Lymphoma, Large-Cell, Anaplastic , Lymphomatoid Papulosis , Lymphoproliferative Disorders , Mycosis FungoidesABSTRACT
Mycosis fungoides (MF) is the most frequent cutaneous T cell lymphoma (CTCL). Since the major tumor cell of MF is the helper T cell, positive markers are usually CD3, CD4 and CD45RO. Some MFs show CD30 positivity and the major differential diagnosis for MF with CD30 positivity includes transformed MF and MF concurrent with primary cutaneous anaplastic large cell lymphoma and lymphomatoid papulosis. As each disease shows a different prognosis, an exact diagnosis is crucial for proper treatment. We now report a case of 44-year-old male patient with mycosis fungoides which developed several papules on preexisting MF patches. On biopsy of the newly formed papules, CD 30 positive cells were observed and the histologic features were consistent with lymphomatoid papulosis. Both the lesions of lymphomatoid papulosis and MF responded well to narrow band ultraviolet B phototherapy.
Subject(s)
Adult , Humans , Male , Biopsy , Diagnosis, Differential , Lymphoma, Primary Cutaneous Anaplastic Large Cell , Lymphoma, T-Cell, Cutaneous , Lymphomatoid Papulosis , Mycosis Fungoides , Phototherapy , PrognosisABSTRACT
Lymphomatoid papulosis (LyP) is a chronic lymphoproliferative disorder characterized by the appearance of crops of papules, nodules, and sometimes large plaques at different stages of development. Pseudocarcinomatous hyperplasia (PCH) presents with extreme proliferation of the epidermis with downgrowth into the dermis, which histologically mimics the features of squamous cell carcinoma. However, squamous cells usually are well differentiated, and atypicalities, such as individual cell keratinization, nuclear hyperplasia, and hyperchromasia, are minimal or absent. PCH has rarely been reported in LyP. Here, we showed that PCH associated with LyP may closely resemble squamous cell carcinoma, thereby giving rise to wrong diagnosis and treatment.
Subject(s)
Carcinoma, Squamous Cell , Dermis , Epidermis , Hyperplasia , Keratins , Lymphomatoid Papulosis , Lymphoproliferative DisordersABSTRACT
Lymphomatoid papulosis (LyP) is a benign, self-healing, papular eruption that can wax and wane over time. Transformation to T-cell lymphoma has been well documented in 10% to 20% of adults with LyP. However, this transformation rarely occurs in patients younger than 20 years of age. Here, we present the first known pediatric patient in Korea, a 12-year-old boy who developed a subcutaneous nodule on the scrotum 13 months after papulonecrotic lesions of LyP were identified on both lower extremities and face. Histological and immunohistochemical examination of the subcutaneous nodule revealed anaplastic large cell lymphoma (ALCL). A T-cell receptor gene rearrangement analysis demonstrated an identical rearranged pattern in the two specimens, indicating that a common T-cell clone had proliferated over time in both the LyP and ALCL lesions.