ABSTRACT
El dermatofibrosarcoma protuberans (DFSP) es un tumor cutáneo, localmente agresivo, que afecta a adultos jóvenes o de edad media. Se han descrito diferentes formas histológicas, siendo la mixoide una de las más infrecuentes. Microscópicamente está constituido por una neoformación que ocupa la dermis e hipodermis, de predominio mixoide, con áreas periféricas conformadas por haces de células fusiformes, de crecimiento infiltrativo, que expresan el marcador inmunohistoquímico CD34 de forma difusa. Presentamos por primera vez un DFSP mixoide en un dedo de la mano izquierda de una niña de 14 años, de más de 10 años de evolución. Se trata del primer caso infantil de DFSP mixoide en dicha localización. Este subtipo histológico se ha descrito fundamentalmente en las extremidades de adultos, siendo excepcional en niños. Se comenta el diagnóstico diferencial con otros tumores mesenquimales mixoides CD34 positivos (AU)
Dermatofibrosarcoma protuberans is a locally aggressive skin tumor that affects young and middle-aged adults. A number of histological variants have been described, the myxoid type being one of the least common. Microscopically it is formed of a neoplastic growth that is located in the dermis and hypodermis and has a predominant myxoid component. Peripherally there are infiltrating bundles of spindle-shaped cells that are diffusely positive for the CD34 immunohistochemical marker. We report a case of myxoid dermatofibrosarcoma protuberans on a finger of the left hand of a 14-year-old girl. The tumor had been present for at least 10 years. This is the first pediatric case of myxoid dermatofibrosarcoma protuberans at this site. This histological subtype has mainly been described on the extremities in adults and is very rare in children. We discuss the differential diagnosis with other CD34+ myxoid mesenchymal tumors (AU)
Subject(s)
Humans , Female , Adolescent , Dermatofibrosarcoma/diagnosis , Dermatofibrosarcoma/pathology , Dermatofibrosarcoma/therapy , In Situ Hybridization, Fluorescence , Immunohistochemistry , Finger Joint , Antigens, CD34 , Myxoma/pathologyABSTRACT
Dermatofibrosarcoma protuberans is a locally aggressive skin tumor that affects young and middle-aged adults. A number of histological variants have been described, the myxoid type being one of the least common. Microscopically it is formed of a neoplastic growth that is located in the dermis and hypodermis and has a predominant myxoid component. Peripherally there are infiltrating bundles of spindle-shaped cells that are diffusely positive for the CD34 immunohistochemical marker. We report a case of myxoid dermatofibrosarcoma protuberans on a finger of the left hand of a 14-year-old girl. The tumor had been present for at least 10 years. This is the first pediatric case of myxoid dermatofibrosarcoma protuberans at this site. This histological subtype has mainly been described on the extremities in adults and is very rare in children. We discuss the differential diagnosis with other CD34(+) myxoid mesenchymal tumors.
Subject(s)
Dermatofibrosarcoma/pathology , Fingers , Adolescent , Female , HumansSubject(s)
Histiocytoma, Benign Fibrous/pathology , Skin Neoplasms/pathology , Adult , Female , HumansABSTRACT
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Subject(s)
Humans , Male , Infant , Mastocytosis, Cutaneous/diagnosis , Skin Diseases, Vesiculobullous/diagnosis , Diagnosis, DifferentialABSTRACT
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Subject(s)
Humans , Male , Middle Aged , Kaposi Varicelliform Eruption/complications , Lymphoma, T-Cell, Cutaneous/complications , Simplexvirus/isolation & purification , Staphylococcus aureus/isolation & purificationABSTRACT
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Subject(s)
Male , Infant, Newborn , Humans , Scabies/complications , Scabies/diagnosis , Scabies/drug therapy , Permethrin/therapeutic use , Pruritus/complications , Staphylococcus aureus/isolation & purification , Streptococcus pyogenes/isolation & purification , Polymerase Chain Reaction/methods , Mite Infestations/diagnosis , Mite Infestations/drug therapy , Scabies/physiopathology , Permethrin/pharmacokinetics , Hyperkeratosis, Epidermolytic/complications , Hyperkeratosis, Epidermolytic/drug therapy , Keratoderma, Palmoplantar/diagnosis , Scabies/epidemiology , Scabies/historySubject(s)
Lymphoma, B-Cell/pathology , Skin Neoplasms/pathology , Skin/pathology , Adult , Atrophy , Humans , MaleABSTRACT
The lack of precise and homogeneous criteria for the recognition of primary cutaneous follicular lymphoma has hindered gaining data on the frequency and clinical and molecular features of this entity. In the course of a review of a series of primary cutaneous lymphoma from different Spanish hospitals, we collected a series of 18 cases of primary cutaneous follicular lymphoma and analyzed its clinical, morphologic, and biologic characteristics. In this review only cases with a follicular pattern of growth, germinal center cytology, and restriction to the skin in a minimum follow-up of 6 months have been included. Cases of primary cutaneous follicular lymphoma were characterized by the expression of classic markers of the germinal center, such as bcl6, CD10, and the presence of aggregates of follicular dendritic cells. They frequently express bcl2 protein, although classical t(14;18) was not found in any of the cases analyzed. Analysis of the bcl6 noncoding first exon showed somatic mutations in two of four cases analyzed, as would be expected in lymphoma deriving from the germinal center. Clinically, most cases showed initial involvement of the head and neck, with relapses in eight cases (involving the skin in five cases, both skin and lymph node in two cases, and lymph node in one case). No death attributable to the tumor was recorded. These data seem to imply that follicular lymphoma may present initially in the skin, lacking the characteristic t(14;18) and having a relatively indolent course. Recognition of these tumors and elucidation of their molecular alterations could lead to properly adapted staging and treatment protocols for these patients.
Subject(s)
Lymphoma, B-Cell/pathology , Lymphoma, Follicular/pathology , Skin Neoplasms/pathology , Adult , Aged , Antigens, Nuclear , Female , Humans , Immunohistochemistry , Lymphoma, B-Cell/genetics , Lymphoma, B-Cell/metabolism , Lymphoma, Follicular/genetics , Lymphoma, Follicular/metabolism , Male , Middle Aged , Mutation/genetics , Neprilysin/metabolism , Nuclear Proteins/metabolism , Polymerase Chain Reaction , Proto-Oncogene Proteins c-bcl-2/genetics , Survival AnalysisABSTRACT
La diabetes insípida central familiar es una enfermedad genética con transmisión autosómica dominante caracterizada por el desarrollo posnatal de un déficit de vasopresina provocado por un déficit en su gen y que se caracteriza clínicamente por la liberación de grandes cantidades de orina diluida (poliuria hipotónica), sed excesiva y polidipsia. Presentamos el caso de una familia afectada de diabetes insípida central familiar, que fue diagnosticada gracias a la detección del caso índice una niña de ocho años que acudió a una revisión rutinaria en atención primaria. Con este artículo se pretende transmitir la importancia del diagnóstico de enfermedades genéticas en el ámbito de la atención primaria de salud y el posterior papel del médico en el consejo genético (AU)
Subject(s)
Female , Child , Humans , Diabetes Insipidus/genetics , Vasopressins/deficiency , Water Deprivation/physiology , Diabetes Insipidus/drug therapy , Osmolar Concentration , Angiotensin II/physiology , Deamino Arginine Vasopressin/therapeutic useABSTRACT
The histogenesis, morphology, immunophenotype, and clinical behavior of cutaneous large B-cell lymphomas (CLBCL) are largely a matter of controversy. We performed an investigation to determine whether CLBCL have features that differentiate them from other large B-cell lymphomas and whether CLBCL is itself a heterogeneous group. To this end, we reviewed the main characteristics of a series of 32 cases of LBCL found in the skin. We reviewed the clinical findings and paraffin sections of the tumors from these 32 patients. The immunohistochemical study performed included p53, MIB1, Bcl2, Bcl6, and CD10 markers. We carried out statistical analysis of these data (univariate and multivariate), seeking an association between the features of the tumors and clinical outcome, as defined by failure-free survival time. Only one patient died as a consequence of the lymphoma. Nevertheless, the accumulated probability of survival without failure at 48 months was 0.46. The number, type, and localization of the lesions were not associated with variations in either survival or failure-free survival. The expression of p53 was negative in this group of CLBCL, whereas Bcl-2 expression or localization in the lower leg did not relate to any other significant feature. Histologic examination of the cases disclosed three different groups: Grade III follicular lymphomas (FLs), monomorphous large B-cell lymphomas (LBCL type I), and LBCL with an admixed component of small B-lymphocytes (LBCL type II). Grade III FL (11 cases) tended to be found in the head and neck and showed CD10 expression in a majority of cases. A higher probability of lymph node relapses was associated with cases located in the head and neck and with CD10+ tumors. Cutaneous large B-cell lymphomas are indolent tumors, but follow an insidious course. Our data support the interpretation that CLBCL is a heterogeneous condition; comprises some LBCL derived from CD10+ germinal center cells which manifests more frequently as tumors in the head and neck region, with an increased probability of relapse in lymph nodes [1] and has some distinctive morphologic features. The existence of a component of small B-cells within the other CLBCL could lend support to the theory that some of these tumors, more than arise de novo, may have originated in preexistent small B-cell lymphomas, but no firm evidence of this is provided in this study.
Subject(s)
Biomarkers, Tumor/analysis , Lymphoma, B-Cell/pathology , Lymphoma, Large B-Cell, Diffuse/pathology , Neoplasm Proteins/analysis , Skin Neoplasms/pathology , Adult , Aged , Aged, 80 and over , B-Lymphocytes/pathology , Combined Modality Therapy , Disease-Free Survival , Female , Follow-Up Studies , Humans , Immunohistochemistry , Immunophenotyping , Lymphoma, B-Cell/chemistry , Lymphoma, B-Cell/mortality , Lymphoma, B-Cell/therapy , Lymphoma, Large B-Cell, Diffuse/chemistry , Lymphoma, Large B-Cell, Diffuse/mortality , Lymphoma, Large B-Cell, Diffuse/therapy , Male , Middle Aged , Neoplasm Staging , Skin Neoplasms/chemistry , Skin Neoplasms/mortality , Skin Neoplasms/therapy , Survival RateABSTRACT
El toxoplama gondii puede causar una infección intrautrina; la incidencia de ésta es de 1:1000 niños nacidos vivos. La toxoplasmosis congénita puede manifectarse como una enfermedad neunatal clinica, una enfermedad durente los primeros meses de vida, como secuelas tardias (Infección recidivante) o una enfermedad subclinica (La más común); del 70 al 90 porciento de los recién nacidos infectados son asintomáticos. Se describe el caso de una lactante menor, atendida en el "Hospital Santa Cruz", Caja Petrolera de Salud , por Decreto Supremo 24303, por presentar succión débil, vómitos, fiebre, hipotonia y coriorretinitis. Laboratorialmente , anemia, linfocitosis, eosinofilia e hiperbilirrubinemia directa; en el liquido cefaloraquidio, xantocromia, pleocitosis mononuclear y un nivel muy alto de proteinas. Tomograficamente, calcificaciones intracraneanas e hidrocefalea supratentorial con presencia de atrofia córtica - subcortical. Serologia: IgM - Elisa, para toxopalsmosis: "Positiva"; IgG - Elisa , para toxoplasmosis , con un valor de 785.3. El tratamiento se inicia con pirimetamina y sulfadiazina; ademas se administra leucovorina y prednisona. Despues de catorce días de internación, la madre de la paciente, informada sobre el pronóstico de la enfermedad, solicita el alta de la lactante, a pesar de la indicación médica de permanecer en el hospital, por mayor tiempo. La paciente no vuelve a controles. Para prevenir la toxoplasmosis congénita, las mujeres embarazadas deben evitar la ingesta de carne gruda, huevos crudos o leche no pasteurizada y la exposición a los receptáculos donde defecan los gatos y a las heces de estos animales. Deben lavar los vegetales;comer frutas peladas; usar guantes durante la preparación de los alimentos y al realizar las tareas de jardineria; evitar el contacto con gatos.
Subject(s)
Humans , Infant , Toxoplasmosis, Congenital/physiopathology , Toxoplasmosis/physiopathology , Chorioretinitis , Hydrocephalus , PyrimethamineABSTRACT
Cutaneous lesions attributed to Leishmania are very seldom observed in classic Kala-Azar, but recently some reports have mentioned them in patients with HIV infection. We found cutaneous lesions whose biopsy disclosed the presence of Leishmania organisms in six patients of a group of 32 HIV patients with visceral Leishmaniasis. These lesions did not present a uniform or specific appearance, even though they tended to localize symmetrically on acral zones. They consisted of erythematous papules and hypopigmented macules on the dorsa of the hands, feet, and elbows; small subcutaneous nodules on the thighs; and erythematoviolaceous, scaly plaques on the face. These lesions accompanied in every case the other symptoms and/or signs of visceral leishmaniasis, responded to anti-leishmanial treatment, and were sometimes the first indicator of recurrence. The histopathological study was non-specific, but showed in every case the presence of abundant amastigotes within the dermal histiocytes and free in the dermis or subcutaneous tissue. Data from literature review are similar to ours.
