ABSTRACT
Abstract Intravascular histiocytosis is a rare condition characterized by the aggregate of histiocytes within dilated dermal vessels. The diagnosis is mainly histophatological and immunohistochemical. We describe a case of a 55 year-old female patient presenting erythematous/purple patches on the breasts, back and limbs. She previously presented ductal carcinoma in the right breast in 2006 which was treated with mastectomy and proceeded to silicone breast implant in 2009. Clinical hypothesis was telangiectatic metastatic carcinoma. Histopathology showed vascular ectasia, thrombosis and recanalization of upper dermis small vessels. On immunohistochemistry, intravascular cells were CD 68+ and negative for estrogen and progesterone receptors, CK7, EMA and AE1/AE3 and endothelial cells were CD64+, leading to the diagnosis of intravascular histiocytosis.
Subject(s)
Humans , Female , Silicones/adverse effects , Histiocytosis/etiology , Histiocytosis/pathology , Skin Diseases, Vascular/etiology , Skin Diseases, Vascular/pathology , Breast Implantation/adverse effects , Immunohistochemistry , Venous Thrombosis/etiology , Venous Thrombosis/pathology , Dilatation, Pathologic , Middle AgedABSTRACT
La histiocitosis eruptiva generalizada, conjuntamente con el xantogranuloma juvenil, constituyen desórdenes histiocíticos de origen dendrítico (también denominados histiocitosis no Langerhans), que comparten características clínico-patológicas e inmunohistoquímicas. Presentamos a una paciente de 3 años de edad con lesiones en la piel clínicamente compatibles con histiocitosis eruptiva generalizada y confirmadas mediante histología e inmunohistoquímica. Luego presentó compromiso en el sistema nervioso central, por lo que fue intervenida quirúrgicamente. En la histopatología de esta lesión, se encontraron células de Touton, compatibles con el diagnóstico de xantogranuloma juvenil. Este caso clínico demuestra la necesidad de considerar estas enfermedades como espectro de una misma entidad.
Both, generalized eruptive histiocytosis and juvenile xanthogranuloma are dendritic histiocytic disorders (also known as non-Langerhans cells histiocytosis) that share clinicopathological and immunohistiochemical characteristics. We present a 3-year-old female patient with skin lesions that were clinically compatible with generalized eruptive histiocytosis, confirmed by histopathological and immunohistochemical studies. During her development the disorder compromised the central nervous system, and surgical intervention of one symptomatic lesion was needed. The histopathological exam of the central nervous system lesion showed Touton cells, compatible with a diagnosis of juvenile xanthogranuloma. This case demonstrates the need to consider these diseases as a spectrum of the same entity.
Subject(s)
Humans , Female , Child, Preschool , Histiocytosis/pathology , Xanthogranuloma, Juvenile/pathology , Histiocytosis/complications , Xanthogranuloma, Juvenile/complicationsABSTRACT
La enfermedad de Rosai-Dorfman, o sinuhistiocitosis con linfadenopatía masiva, es una rara patología que se presenta con linfadenopatía dolorosa generalizada, más frecuentemente en cabeza y cuello. Su curso clínico y tratamiento es variable. Se describe el caso de un paciente masculino con compromiso nasal de la enfermedad...
Rosai-Dorfman disease, or sinus histiocytosis with massive lymphadenopathy, is a rare disorder presented as painless bilateral lymph node enlargement mainly in the head and neck region. Clinical course and treatment regimen are variable. We described one male patient with nasal involment...
Subject(s)
Humans , Neck , Histiocytosis , Histiocytosis/etiology , Histiocytosis/pathology , Histiocytosis/rehabilitationABSTRACT
Introdução: A histiocitose de células de Langerhans é um distúrbio proliferativo de células inflamatórias de etiologia desconhecida. É doença rara da faixa pediátrica. Objetivo: Realizar revisão de literatura sobre a histiocitose de células de Langerhans, focando as manifestações otorrinolaringológicas. Materiais e métodos: A metodologia utilizada foi consulta à base de dados on line MEDLINE, de 1966 a 2008, pesquisando a partir dos termos histiocitose de células de Langerhans, osso temporal e otorrinolaringologia. Revisão da Literatura: As manifestações em cabeça e pescoço são as mais frequentes e seu diagnóstico torna-se difícil uma vez que mimetiza outras doenças mais comuns vistas pelo otorrinolaringologista, como otite externa, mastoidite aguda e gengivite. A doença no osso temporal expressa-se como otorreia de repetição e granulomas de conduto auditivo externo ou retroauricular. A avaliação radiológica evidencia lesões líticas principalmente em calota craniana, mandíbula, osso temporal e costelas. O diagnóstico definitivo é feito por biópsia através dos achados histopatológicos e detecção pela imunohistoquímica do antígeno CD1a. A principal forma de tratamento é a quimioterapia e, em menor escala, radioterapia ou cirurgia. Conclusões: Manifestações otorrinolaringológicas devem ser suspeitadas por sintomas otológicos recorrentes e pela presença de tecido de granulação retroauricular ou de conduto auditivo externo. A biópsia com achados histológicos característicos e imunohistoquímica positiva para CD1a são diagnósticos. A quimioterapia pode ser o tratamento inicial na maioria dos casos ou adjuvante nas formas refratárias ou recorrentes.
Introduction: The Langerhans cell histiocytosis is an inflammatory cells proliferative disorder of unknown etiology. It is uncommon disease in children. Objective: To proceed with a literature review on Langerhans cell histiocytosis, with focus on the otorhinolaryngological complications. Materials and methods: The methodology used was advised based on online data from MEDLINE, between 1966 and 2008, with research of terms related to Langerhans cell histiocytosis, temporal bones and otorhinolaryngology. Literature Review: The manifestations in the head and neck are the most common ones and their diagnosis becomes difficult once it mimetizing other more common diseases the otorhinolaryngologist sees as external ear eczema, acute mastoiditis and gingivitis. The temporal bone disease manifests as recurrent otorrhea and external auditory meatus and retroauricular granulomas. The radiological evaluation confirms lytic lesions especially in the cranial cap, jaw, temporal bones and spines. The definitive diagnosis is made by biopsy through the histopathological discoveries and immunohistochemistry detection of the CD1a antigen. The main form of treatment is by chemotherapy and, in a lower scale, radiotherapy or surgery. Conclusions: he otorhinolaryngological manifestations must be suspected for recurrent otological symptoms and the presence of retroauricular granulation tissue or and the external auditory meatus. The biopsy with characteristic histological discoveries and immunohistochemistry positive for CD1a were diagnostic. The chemotherapy may be the initial treatment in most cases or secondary in refractory or recurrent forms.
Subject(s)
Humans , Histiocytosis/pathology , Immunohistochemistry , Langerhans Cells , Temporal Bone/pathology , Otorhinolaryngologic Diseases/diagnosisABSTRACT
Las histiocitosis son un grupo heterogéneo de enfermedades unidas débilmente por características microscópicas y con una terminología que se sobrepone. Muchos de los desórdenes histiocíticos tienen su inicio en la infancia o niñez. Esta revisión se enfocará en estos síndromes histiocíticos de la infancia.
Subject(s)
Humans , Female , Infant, Newborn , Infant , Skin Diseases/pathology , Histiocytosis, Langerhans-Cell/diagnosis , Histiocytosis, Langerhans-Cell/pathology , Histiocytosis/classification , Histiocytosis/diagnosis , Histiocytosis/pathologyABSTRACT
Histiocytoses are an important group of dermatoses characterized by histological infiltrates predominantly rich in cells of monocyte-macrophage origin. The present review focuses on the salient clinical, diagnostic and therapeutic features of different entities included in this group
Subject(s)
Histiocytosis/pathology , Histiocytosis/therapy , PrognosisABSTRACT
A 42-year-old man with Erdheim-Chester disease (EC) is presented. This is the first case of this disease reported in Korea. The patient complained of knee pain and plain roentgenogram of the bilateral legs revealed diffusely increased density, coarsened trabecular pattern, and cortical thickening in the diaphysis, and metaphysis as well as epiphysis. Magnetic resonance imaging revealed that the lesions showed low signal intensity on T1-weighted images and heterogeneously low and high signal intensity on T2-weighted images. Histological examination of the biopsy specimen showed a xanthogranulomatous lesion consisting aggregations of foamy histiocytes and Touton-type giant cells. Immunohistochemical staining showed positive reaction to anti-S-100 and lysozyme in the cytoplasm of the giant cells.
Subject(s)
Adult , Humans , Male , Bone Marrow/pathology , Histiocytosis/pathology , Knee/diagnostic imaging , Knee/pathology , Magnetic Resonance Imaging , Osteosclerosis/diagnostic imaging , Osteosclerosis/pathologyABSTRACT
El granuloma eosinofilico es una forma localizada de histiocitosis X o histiocitosis de células de langerhans caracterizada por lesiones líticas de uno o mas huesos. El siguiete es el reporte de un paciente masculino de dieciocho meses de edad con otorrea izquierda de ocho meses de evolución, tejido polipoide en conducto auditivo externo izquierdo y aumento de volumen de tejidos blandos en región retroauricular. A la palpación era evidente un desnivel en la cortical de mastoides del mismo lado. Radiológicamente se apreciaban lesiones líticas de hueso temporal izquierdo, con preservación de cadena osicular y falta de neumatización de la mastoides. El reporte patológico definitivo fue de granuloma eosinofilico
Subject(s)
Humans , Male , Infant , Biopsy , Eosinophilic Granuloma/diagnosis , Eosinophilic Granuloma/pathology , Eosinophilic Granuloma , Histiocytosis/diagnosis , Histiocytosis/pathology , Temporal Bone/pathology , TomographyABSTRACT
Presentamos el caso de un paciente de 44 años de edad con Reticulohistiocitosis Multicentrica, quien evidencias la enfermedad con aparición de erupción nodular cutánea y poliartritis, realizándose el diagnóstico al evaluar lesiones cutáneas y líquido sinovial. El paciente sometido a tratamiento con anti-inflamatorios esteroideos y agentes alquilantes, evidenciandose remisión de la fase aguda de la enfermedad pero con progresión en la destrucción de las articulaciones previamente afectadas
Subject(s)
Humans , Male , Arthritis, Rheumatoid/diagnosis , Arthritis, Rheumatoid/therapy , Biopsy , Histiocytosis/diagnosis , Histiocytosis/pathology , Lymphatic Diseases/diagnosisABSTRACT
To study the hematological and histochemical features of malignant histiocytosis cases diagnosed at King Khalid University Hospital. This is a retrospective analysis of malignant histiocytosis cases diagnosed by bone marrow examinations at the Hematology Section, Department of Pathology, King Khalid University Hospital. The medical records were evaluated for clinical symptoms and signs at presentation and peripheral blood count. Bone marrow aspirates and biopsies were reviewed for morphological features. Immunohisto-chemical staining of bone marrow trephine biopsies was performed. Over 11 years, from February 1984 to March 1995, four cases of malignant histiocytosis were diagnosed at King Khalid University Hospital [KKUH] in Riyadh. They were two males and two females. The age range was 25-56 years. The predominant symptoms at presentation were fever and fatigability. All patients had hepatomegaly and three had splenomegaly. One patient had localized lymphadenopathy and skin involvement. Three patients had pancytopenia while one patient had anemia, thrombocytopenia and leukocytosis. Immunohistochemical stains on three cases showed positivity with 1 antitrypsin and lysozyme. One case showed CDS [T cell marker] positivity by flow cytometry. The clinical, hematological and immunohistochemical staining findings are described
Subject(s)
Humans , Male , Female , Histiocytosis/pathology , Bone Marrow Examination/methods , Bone Marrow/anatomy & histologyABSTRACT
Se expone el caso de un varón de 36 años de edad, que consultó por presentar una erupción de lesiones nodulares diseminadas en tronco y miembros inferiores, de dos meses de evolución, que se acompañaba de sudoración, fiebre y mal estado general. La histología cubría los requisitos descriptos por Flynn, para la denominada histiocitosis atípica regresiva. Las lesiones regresaron espontáneamente en dos meses. Los estudios de inmunohistoquímica demostraron fenotipo T, y 60 por ciento de positividad para el antígeno Ki1, lo que confirma que este cuadro corresponde a formas autoregresivas de linfomas cutáneos anaplásicos de grandes células Ki1 positivos
Subject(s)
Humans , Male , Adult , Histiocytosis/diagnosis , Lymphoma, T-Cell, Cutaneous/diagnosis , Betamethasone , Betamethasone/therapeutic use , Diagnosis, Differential , Histiocytosis/pathology , Lymphoma, T-Cell, Cutaneous/pathologyABSTRACT
Clinical and pathological findings in a case of necrotizing lymphadenitis are described. Histologically the necrotic areas show striking karyorrhexis, absence of polymorphonuclear granulocytes and surrounding zones of large reactive lymphoid cells. The disorder must be carefully distinguished from a lymphoma which it may resemble closely.