ABSTRACT
INTRODUCCIÓN: El angioma en penacho o tufted angioma (TA) es una tumoración vascular benigna poco frecuente, que suele aparecer en la infancia, aunque existen casos de aparición en la edad adulta. Su presentación clínica es muy variable. Se manifiesta típicamente como una mácula, pápula o nódulo eritematovioláceo en el tronco o el cuello. Histológicamente, se caracteriza por agregados de lóbulos angiomatosos en la dermis formando pequeños penachos de capilares. PACIENTES Y MÉTODOS: Estudio retrospectivo observacional de los casos diagnosticados de TA en los últimos 20 años en nuestro centro. RESULTADOS: Presentamos un total de 9 casos de angiomas en penacho en la infancia. El 77,7% de los casos fueron congénitos, lo que representa una frecuencia superior a la descrita previamente. Nuestros pacientes presentaron regresión espontánea en el 55,5% de los casos, siendo más frecuente en el grupo de TA congénitos. A diferencia de las otras series descritas en la literatura, observamos un mayor porcentaje de pacientes con regresión espontánea, un predominio femenino (6 de los 9 niños) y una localización más frecuente en miembros los superiores. Ninguno de nuestros pacientes presentó fenómeno de Kasabach-Merritt. CONCLUSIONES: Dada la alta tasa de involución espontánea en TA congénitos o tempranos, en ausencia de otras complicaciones, la vigilancia sería una buena opción de manejo, monitorizando estrechamente al paciente
INTRODUCTION: Tufted angioma (TA) is a rare benign vascular tumor that mostly appears during infancy or early childhood, although there are cases reported in adults. Clinical presentation and evolution of TA can vary. Histologically, it takes on a classic appearance of vascular tufts ('cannon ball' like appearance). PATIENTS AND METHODS: A retrospective observational study was conducted that included all patients diagnosed with TA at our center in the last 20 years. Results A series of 9 cases of tufted angioma in childhood are presented, 77.7% of which were congenital. This represents a frequency higher than previously described. Spontaneous regression was observed in 55.5% of the cases, and was more frequent in the congenital TA group. Unlike other TA series reported in the literature, a higher proportion of patients with spontaneous regression was observed in this series, with a higher prevalence in females (6 out of 9 children) and predominantly located in the upper limbs. None of our patients had Kasabach-Merritt phenomenon. CONCLUSIONS: There are many ways of treating TA, but none are uniformly effective. Given the high rate of spontaneous regression in congenital or early TA, we suggest that, in the absence of other complications, monitoring would be a good option for management
Subject(s)
Child , Child, Preschool , Female , Humans , Infant , Infant, Newborn , Male , Hemangioma, Capillary/diagnosis , Hemangioma, Capillary/drug therapy , Hemangioma, Capillary/pathology , Hemangioma, Capillary/complications , Kasabach-Merritt Syndrome , Aspirin/therapeutic use , Propranolol/therapeutic use , Prednisone/therapeutic use , Epidemiological Monitoring/trends , Neoplasms, Vascular Tissue/pathology , Congenital Abnormalities/pathology , Vascular System Injuries/pathology , Hemangiopericytoma , Sarcoma, Kaposi , Hemangioendothelioma , Diagnosis, Differential , Retrospective Studies , Observational Study , Spain/epidemiologyABSTRACT
INTRODUCTION: Tufted angioma (TA) is a rare benign vascular tumor that mostly appears during infancy or early childhood, although there are cases reported in adults. Clinical presentation and evolution of TA can vary. Histologically, it takes on a classic appearance of vascular tufts ("cannon ball" like appearance). PATIENTS AND METHODS: A retrospective observational study was conducted that included all patients diagnosed with TA at our center in the last 20 years. RESULTS: A series of 9 cases of tufted angioma in childhood are presented, 77.7% of which were congenital. This represents a frequency higher than previously described. Spontaneous regression was observed in 55.5% of the cases, and was more frequent in the congenital TA group. Unlike other TA series reported in the literature, a higher proportion of patients with spontaneous regression was observed in this series, with a higher prevalence in females (6 out of 9 children) and predominantly located in the upper limbs. None of our patients had Kasabach-Merritt phenomenon. CONCLUSIONS: There are many ways of treating TA, but none are uniformly effective. Given the high rate of spontaneous regression in congenital or early TA, we suggest that, in the absence of other complications, monitoring would be a good option for management.
Subject(s)
Hemangioma/diagnosis , Skin Neoplasms/diagnosis , Child , Female , Hemangioma/therapy , Humans , Infant , Infant, Newborn , Male , Neoplasm Regression, Spontaneous , Retrospective Studies , Skin Neoplasms/therapyABSTRACT
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Subject(s)
Humans , Male , Female , Infant , Child , Nevus/diagnosis , Osteopoikilosis/diagnosis , Connective Tissue Diseases/diagnosis , beta-Thalassemia/complications , Diagnosis, DifferentialSubject(s)
Osteopoikilosis , Skin Diseases, Genetic , Child , Child, Preschool , Female , Humans , Male , Osteopoikilosis/diagnosis , Skin Diseases, Genetic/diagnosisSubject(s)
Influenza A virus/pathogenicity , Influenza, Human/diagnosis , Ulcer/etiology , Vulvar Diseases/diagnosis , Adolescent , Antibodies, Viral/blood , Diagnosis, Differential , Female , Herpes Genitalis/diagnosis , Humans , Influenza A virus/immunology , Influenza, Human/blood , Influenza, Human/complications , Vulvar Diseases/etiologyABSTRACT
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Subject(s)
Male , Infant, Newborn , Humans , Ectodermal Dysplasia/complications , Scalp/pathology , Abnormalities, MultipleSubject(s)
Leukemia, Myelomonocytic, Acute/diagnosis , Leukemic Infiltration/diagnosis , Sarcoma, Myeloid/etiology , Skin/pathology , Antigens, CD/analysis , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Combined Modality Therapy , Hematopoietic Stem Cell Transplantation , Humans , Infant , Leukemia, Myelomonocytic, Acute/drug therapy , Leukemia, Myelomonocytic, Acute/pathology , Leukemia, Myelomonocytic, Acute/surgery , Male , Transplantation, HomologousABSTRACT
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Subject(s)
Male , Infant , Humans , Leukemia, Myeloid, Acute/pathology , Sarcoma, Myeloid/pathology , Neoplasm Invasiveness/pathologyABSTRACT
Phacomatosis pigmentokeratotica is a rare syndrome defined by the association of an organoid nevus occasionally with sebaceous differentiation, a speckled lentiginous nevus, and other extracutaneous anomalies. The disorder is a consequence of the so-called twin spot genetic mechanism. We describe the first occurrence involving malignant degeneration of both nevus components, giving rise to three basal cell carcinomas over the sebaceous nevus and a malignant melanoma of the superficial spreading type over the speckled lentiginous nevus. This observation, in concert with the other instances reported in the literature, points to the need for adequate patient follow-up to ensure early detection and treatment of any possible associated malignant degeneration.
Subject(s)
Carcinoma, Basal Cell/physiopathology , Neurocutaneous Syndromes/physiopathology , Nevus, Pigmented/physiopathology , Skin Neoplasms/physiopathology , Adult , Carcinoma, Basal Cell/complications , Cell Transformation, Neoplastic , Disease Progression , Follow-Up Studies , Humans , Male , Melanoma/complications , Melanoma/physiopathology , Neurocutaneous Syndromes/complications , Nevus, Pigmented/complications , Skin Neoplasms/complicationsABSTRACT
Lymphangioma circumscriptum is an uncommon hamartomatous malformation that is not only localized to an area of skin, but also spreads to subcutaneous tissue and muscle (Whimster's hypothesis). We describe a 7-year-old boy with a bluish plaque on the left side of the back and abdomen with clusters of translucent vesicles (frog spawn). Magnetic resonance imaging showed the lymphangioma had spread to muscles and even fat adjacent to the left kidney. We decided against radical surgery as it would have been too aggressive, and limited excision can produce new surface lymphangiomas.
Subject(s)
Lymphangioma/pathology , Skin Neoplasms/pathology , Abdomen , Back , Child , Humans , Lymphangioma/surgery , Magnetic Resonance Imaging , Male , Muscle Neoplasms/diagnostic imaging , Muscle Neoplasms/secondary , Muscle Neoplasms/surgery , Radiography , Skin Neoplasms/surgery , Treatment OutcomeABSTRACT
Presentamos el caso de una niña de 6 años de edad con lesiones hiperqueratósicas distribuidas siguiendo las líneas de Blaschko en todo el hemicuerpo izquierdo. La biopsia cutánea demostró un patrón de hiperqueratosis epidermolítica. Se trató con acitretino a dosis de 0,5 mg/ kg/d con buena respuesta. Recordamos la importancia de este mosaicismo por la posibilidad de tener hijos afectados de eritrodermia ictiosiforme congénita ampollosa en la descendencia. (AU)
Subject(s)
Female , Child , Humans , Hyperkeratosis, Epidermolytic/drug therapy , Acitretin/pharmacology , Acitretin/administration & dosage , Mosaicism/genetics , Mosaicism/diagnosis , Hyperkeratosis, Epidermolytic/diagnosis , Hyperkeratosis, Epidermolytic/geneticsABSTRACT
The Neutrophilic dermatosis (ND) is considered as an independent entity with diverse clinical manifestations among which there are: gangrenous pyoderma, nodous erythema, Sweets Syndrome, vesiculopustula eruptions associated to ulcerous colitis and intestinal short circuit syndrome with or without short circuit. Histologically, they are characterized by infiltration of polymorphonuclear neutrophils, generally at the dermic level, but also at the epidermic. They are usually associated to systemic diseases, especially to chronic intestinal inflammatory disease. Our aim was to describe two forms of clinical presentation of neutrophilic dermatosis: gangrenous pyoderma and vesiculopustula eruption, associated to ulcerous colitis starting at advances ages.
Subject(s)
Colitis, Ulcerative/complications , Neutrophils , Skin Diseases/etiology , Aged , Aged, 80 and over , Female , Humans , Middle Aged , Skin Diseases/pathologyABSTRACT
Skin involvement in non-Hodgkin lymphoma is usually specific and may precede systemic manifestations of the disease by months or even years. We report the case of a man with an inguinal plaque lesion of 1 year's evolution, diagnosed as polymorphous lymphoplasmocytoid lymphoma (immunocytoma). The evaluation of spread only revealed mesenteric lipodystrophy, whose association with lymphoid processes is more common than would be expected.
Subject(s)
Leukemia, Lymphocytic, Chronic, B-Cell/complications , Leukemia, Lymphocytic, Chronic, B-Cell/pathology , Lipodystrophy/complications , Lipodystrophy/pathology , Mesentery/pathology , Skin Neoplasms/complications , Skin Neoplasms/pathology , Aged , Humans , Lymphoma, Non-Hodgkin/pathology , Male , Peritoneal Diseases/complications , Peritoneal Diseases/pathologyABSTRACT
Sweet's syndrome, or febrile neutrophilic dermatosis, is a disease first described by Sweet R.D. in 1964 as a dermatologic disease. Subsequently, it has been associated to several disease. One of those rarely describe is the association to chronic intestinal inflammatory disease. We reviewed the cases studied in our hospital since 1980 and found two cases associated to chronic intestinal inflammatory disease. We recommend the carrying out of gastrointestinal studies in patients afflicted by Sweet's syndrome to detect its association.
Subject(s)
Inflammatory Bowel Diseases/diagnosis , Leukocytosis/diagnosis , Neutrophils , Skin Diseases/diagnosis , Acute Disease , Adult , Colitis, Ulcerative/diagnosis , Crohn Disease/diagnosis , Female , Humans , Middle Aged , SyndromeABSTRACT
Kaposi's sarcoma is frequently associated to other primary neoplasias, especially those arising from mononuclear-phagocytic system. A case of Kaposi's sarcoma associated to Hodgkin's disease is presented.
Subject(s)
Hodgkin Disease/complications , Sarcoma, Kaposi/complications , Skin Neoplasms/complications , Adult , Disease Susceptibility , Female , Hodgkin Disease/diagnosis , Hodgkin Disease/immunology , Humans , Sarcoma, Kaposi/immunology , Skin Neoplasms/immunologyABSTRACT
We present a 45 years old male with dermatomyositis refractory to eight months of standard treatment who responded to plasma exchange. The efficacy of the procedure is estimated from clinical follow-up (increase in strength) and serum creatine-kinase activity (decrease from 3,380 to 44 u/ml.) after five sessions of plasma exchange. The therapeutic indications for PE are reviewed in dermatological diseases with emphasis on cases where the response to steroids or cytotoxic drugs is inadequate.
