ABSTRACT
Primary cutaneous anaplastic large cell lymphoma (C-ALCL) is a rare subtype of primary cutaneous lymphoma with a favorable prognosis. Primary cutaneous CD30+ lymphoproliferative disorders, which include C-ALCL and lymphomatoid papulosis, are the second most common group of cutaneous T-cell lymphomas. C-ALCL is comprised of large cells with anaplastic, pleomorphic, or immunoblastic cytomorphology, and indeed, more than 75% of the tumor cells express the CD30 antigen. C-ALCL clinically presents with solitary or localized reddish-brown nodules or tumors, and sometimes indurated papules, and they may be with ulceration covering with dark eschar. Multifocal lesions are seen in 20% of the patients. Extracutaneous dissemination, which mainly involves the regional lymph nodes, occurs in 10% of patients. A 69-year-old man noticed a mild elevated cutaneous lesion containing central ulceration covering with brownish black necrotic tissue on the right lower lip, and the lesion was surgically removed. After the first operation, another skin lesion was developed and the histological examination confirmed the diagnosis, C-ALCL. Eight specimens were excised during the 7-month follow-up period. The patient started the treatment with low-dose oral methotrexate (15 mg/wk) and there was no recurrence for 11 months.
Subject(s)
Aged , Humans , Ki-1 Antigen , Diagnosis , Follow-Up Studies , Lip , Lymph Nodes , Lymphoma , Lymphoma, Primary Cutaneous Anaplastic Large Cell , Lymphoma, T-Cell, Cutaneous , Lymphomatoid Papulosis , Lymphoproliferative Disorders , Methotrexate , Prognosis , Recurrence , Skin , UlcerABSTRACT
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-LymphocytesSubject(s)
Humans , Female , Adult , Sarcoidosis/complications , Sarcoidosis/pathology , Skin Neoplasms/etiology , Skin Neoplasms/pathology , Lymphomatoid Papulosis/etiology , Lymphomatoid Papulosis/pathology , Skin/pathology , Skin Neoplasms/immunology , Biopsy , Immunohistochemistry , Risk Factors , Lymphomatoid Papulosis/immunology , Fatal OutcomeABSTRACT
CD30+ lymphoproliferative disorders (LPD) represent a spectrum of T-cell lymphoma including lymphomatoid papulosis and anaplastic large cell lymphoma (ALCL). Epidermis overlying cutaneous CD30+ LPD often shows epidermal hyperplasia, hyperkeratosis, crusting, and ulceration and it is difficult to distinguish from carcinoma such as keratoacanthoma (KA) or squamous cell carcinoma (SCC). Several cases of pseudocarcinomatous hyperplasia mimicking KA or SCC in CD30+ LPD have been reported. The relationship between CD30+ LPD and epithelial proliferations has not yet well understood. It was reported that a variety of mediators, including epidermal growth factor (EGF), transforming growth factor-α and EGFR from CD30+ LPD could attribute to epidermal hyperplasia. However, separate and distinct SCC occurring in CD30+ LPD has rarely been reported. Herein, we present a rare case of coexistence of SCC and cutaneous ALCL located on the same region.
Subject(s)
Carcinoma, Squamous Cell , Epidermal Growth Factor , Epidermis , Epithelial Cells , Hyperplasia , Keratoacanthoma , Lymphoma , Lymphoma, Large-Cell, Anaplastic , Lymphoma, Primary Cutaneous Anaplastic Large Cell , Lymphoma, T-Cell , Lymphomatoid Papulosis , Lymphoproliferative Disorders , UlcerABSTRACT
La presencia de verrugas anogenitales en niños es una situación controvertida, ya que las vías de acceso del virus del papiloma humano (HPV) al área anogenital infantil pueden ser la perinatal, a partir de lesiones en el canal del parto,la transmisión por auto o heteroinoculacióndes de verrugas vulgares en las manos de los propios niños, familiares o cuidadores, y por abuso sexual. Por ello, para llegar a un correcto diagnóstico y tratamiento es importante contar con una buena historia clínica. Presentamos el caso de una paciente en edad escolar, de sexo femenino, que tras un abuso sexual, presentó una infección por elvirus de la inmunodeficiencia humana (VIH) y desarrolló una papulosis bowenoide.
The presence of anogenital warts in childrenis a controversial situationsincethepaths of human papillomavirus (HPV) in a child´sanogenitalareamay be acquiredperinatally, fromlesions in thebirth canal oracquiredviaauto orheteroinoculationfromcommonwartsonthehands of childrenthemselves, familymembersorcaregivers and fromthegenitals of adultsduring sexual abuse. Hencetheimportance of a goodhistorytoreach a correct diagnosis and treatment.Wereportthe case of a femalepatient,whoafterbeingsexuallyabused, developed HIV and a bowenoidpapulosis.
Subject(s)
Humans , Female , Child , Child Abuse, Sexual , Condylomata Acuminata , Papillomavirus Infections , Lymphomatoid Papulosis , Acquired Immunodeficiency Syndrome , Uterine Cervical NeoplasmsABSTRACT
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
La papulosis linfomatoide (PL) es considerada en la actualidad una forma indolente de linfoma cutáneo CD30+. Su presentación es más frecuente entre la 4º y 5º décadas de la vida, con un discreto predominio en el sexo masculino (1,5/1). Su mecanismo etiopatogénico es complejo y se ha vinculado principalmente con factores genéticos e inmunitarios. Aunque exhibe características clínicas de benignidad, se manifiesta histológicamente con rasgos de malignidad. Las técnicas inmunohistoquímicas resultan de utilidad a los fines diagnósticos y recientemente han permitido la identificación de un nuevo tipo de PL que remeda un linfoma cutáneo primario agresivo a células T epidermotrópico CD8+ (propuesto como PL tipo D). Puede hallarse asociada a otros trastornos linfoproliferativos y a entidades inflamatorias con perfil de citocinas Th2, entre otros. El diagnóstico diferencial con otros linfomas cutáneos, y especialmente con los casos que presentan el antígeno CD30, puede a veces resultar muy dificultoso. Si bien la PL tiene un curso clínico benigno, quienes la padecen tienen un mayor riesgo de desarrollar una segunda neoplasia. Las opciones terapéuticas disponibles son múltiples; sin embargo, ninguna de ellas ha resultado hasta ahora completamente eficaz.
Subject(s)
Humans , Lymphoma, T-Cell/classification , Lymphoma, T-Cell/pathology , Lymphoma, T-Cell/therapy , Lymphomatoid Papulosis/genetics , Lymphomatoid Papulosis/pathology , /analysis , Diagnosis, Differential , Immunohistochemistry , Skin Neoplasms/genetics , Prognosis , Lymphoproliferative Disorders/genetics , Lymphoproliferative Disorders/pathologyABSTRACT
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.
Subject(s)
Adult , Child , Humans , Clone Cells , Genes, T-Cell Receptor , Korea , Lower Extremity , Lymphoma, Large-Cell, Anaplastic , Lymphoma, T-Cell , Lymphomatoid Papulosis , Scrotum , T-LymphocytesABSTRACT
Lymphomatoid papulosis (LyP) and primary cutaneous anaplastic large cell lymphoma (ALCL) are grouped under the category of cutaneous T-cell lymphomas as CD30+ lymphoproliferative disorders. Though LyP is clinically characterized by chronic recurrent papulonodular cutaneous lesions, it shows malignant features on the histologic findings. LyP is associated with lymphomas, including primary cutaneous ALCL, mycosis fungoides and other lymphoproliferative disorders. A few cases of LyP related to ALCL have been reported in Korea. We report here on an interesting case of primary cutaneous ALCL that developed in succession after LyP in a 38-year-old female.