Nevus azul celular parotídeo, una tumoración inusual / Parotid cellular blue nevi, an unusual mass

Introducción: el nevus azul celular es una tumoración melanocítica dérmica benigna. En ocasiones, puede ser falsamente diagnosticada como lesiones malignas, entre ellas, el melanoma. Caso clínico: se trata de una mujer de 37 años que presentó una masa parotídea izquierda de cuatro meses de evolución correspondiente con un nevus azul celular. Discusión: la región de la cabeza y cuello es la tercera en frecuencia, tras la sacrococcígea y las extremidades. Ante una tumoración melanocítica, es importante la confirmación diagnóstica, debido a las similitudes, tanto clínicas como anatomopatológicas, del nevus azul celular con el melanoma maligno. Conclusiones: es muy importante el diagnóstico diferencial correcto, para lo cual es de ayuda el uso de las tinciones inmunohistoquímicas. El tratamiento de esta tumoración es la exéresis quirúrgica con márgenes, esto presenta un comportamiento benigno y baja tasa de recidiva.

Introduction: Cellular blue nevi is a benign dermal melanocytic tumor. Occasionally, it can be falsely diagnosed as malignant lesions, including melanoma. Clinical case: This is a 37-year-old woman who presented with a left parotid mass of four months of evolution, corresponding with a cellular blue nevi. Discussion: The region of the head and neck is the third in frequency, after the sacrococcygeal and the extremities. During the study of a melanocytic tumor, diagnostic confirmation with a biopsy is important, due to the similarities, both clinical and pathological, of cellular blue nevi with malignant melanoma. Conclusions: the correct differential diagnosis is very important, for which immunohistochemical study is helpful. The treatment of this tumor is the surgical excision with margins, presenting benign behaviour and low recurrence rate.

An extensive intraoral blue nevus in an elder patient.

OBJECTIVE: To report a rare case of intraoral extensive blue nevus in an elder patient. BACKGROUND: Oral blue nevi is a well-recognised skin melanocytic neoplasm that rarely occurs in the oral cavity and may mimic melanoma in the early stages. METHODS: An incisional biopsy was performed, and the diagnosis was blue nevus. CONCLUSION: Both the clinician and pathologist must be aware of such a presentation to diagnose and treat appropriately.

Nevus azul celular con satelitosis y velo azul blanquecino simulando un melanoma, a propósito de un caso / Cellular blue nevus with satellitosis and blue veil whitish simulating a melanoma, purpose of a case

Los nevi azules son proliferaciones melanocíticas dendríticas benignas a nivel dérmico, congénitos o adquiridos, debido a un defecto migratorio embrionario de melanocitos a partir de la cresta neural. Se manifiestan clásicamente como una pápula, nódulo o placa de color azul o azul-gris. Muchos subtipos histológicos se han descrito, siendo los más comunes el nevus azul común, nevus azul celular y nevus azul combinado. Las formas esporádicas incluyen al nevus azul lineal, eruptivo, agminado y con satelitosis. La dermatoscopía característica muestra un patrón de pigmentación homogéneo monocromático azul o azul-grisáceo, con ausencia de otras estructuras. Sin embargo, se han descrito también patrones de pigmentación dicromáticos y multicromáticos, además de estructuras tales como red de pigmento, puntos, glóbulos, proyecciones radiadas, pseudópodos, áreas cicatriciales blanquecinas, patrón vascular y rosetas. El diagnóstico diferencial de los nevi azules incluye lesiones melanocíticas y no melanocíticas, benignas y malignas, destacando entre ellas el melanoma. Se presenta el caso de un paciente de sexo masculino de 30 años, portador de un nevus azul celular con cambios de rápida evolución, con desarrollo de lesiones satélites y un aspecto dermatoscópico sugerente de malignidad, simulando un melanoma.

Blue nevi are benign, congenital, or acquired, dermal dendritic melanocytic proliferations due to an embryonic migratory defect of melanocytes starting from the neural crest. They classically manifest as a blue or blue-gray papule, nodule, or plaque. Many histological subtypes have been described, including common blue nevus, cellular blue nevus and combined blue nevus. Sporadic forms include linear blue nevus, eruptive, agminate and with satellitosis. Characteristic dermoscopy shows a homogeneous monochromatic blue or steel-blue pigmentation pattern, with the absence of other structures. However, dichromatic and multichromatic pigmentation patterns have also been described, in addition to structures such as pigment network, dots, globules, streaks, pseudopods, whitish scar areas, vascular pattern and rosettes. Differential diagnosis of blue nevi includes melanocytic and non-melanocytic, benign and malignant lesions, most notably melanoma. The case of a 30-year-old male patient is presented, with a cellular blue nevus with rapidly evolving changes, with development of satellite lesions and a dermoscopic appearance suggestive of malignancy, mimicking melanoma.

Síndrome de Blue Rubber Bled Nevus. Presentación de un caso / Syndrome of Blue Rubber Bled Nevus. Case presentation

RESUMEN El síndrome de Blue Rubber Bled Nevus se caracteriza por múltiples malformaciones cutáneas, asociadas con malformaciones menores en órganos internos, (comúnmente afectan los órganos del sistema digestivo y el sistema nervioso central). A nivel mundial, se afirma que hay cerca de 1 a 2 millones de individuos afectados por este síndrome, y se cree que incide en 1 de cada 6000. En recién nacidos la incidencia se ha calculado en menos de 1 caso por 100 000 persona/año. Se presentó un caso de una paciente femenina de 8 años de edad, con antecedentes de hemangiomas múltiples de color azul violáceo, distribuidos por toda la superficie corporal, de diferentes diámetros. Algunos protruyen y depresibles al tacto. Presentaba ptosis palpebral y movimientos torpes de los cuatro miembros, epilepsia y afectaciones del neurodesarrollo el cual corresponde con una edad cronológica de 11 meses. Mostraba una disminución importante de la visión. Presencia de pie valgo a predominio derecho. Apareció anemia leve por déficit de hierro, inmunoglobulina C3 y C4, la química sanguínea al límite de lo normal. La resonancia magnética nuclear mostró importantes signos de atrofia cortical, más acentuados en región temporal derecha. Son entidades de muy escasa presentación y en la provincia de Matanzas es el único caso con este diagnóstico, por lo que constituyó el motivo para la presentación del caso (AU).

ABSTRACT The syndrome of Blue Rubber Bled Nevus is characterized by multiple skin malformations, associated to minor malformations in internal organs (it commonly affects the organs of the digestive system and the central nervous system). It is affirmed that around 1 to 2 millions of individuals are affected by this syndrome worldwide, and it is believed that it has incidence on 1 of each 6000 persons. In newborns the incidence has been calculated in less than 1 case per 100 000 persons/year. The authors presented the case of a female patient, aged 8 years, with antecedents of purplish-blue multiple hemangiomas of different diameters, distributed all over the surface if the body. Several of them are protruding and depressible to the touch. She presented eyelid ptosis and slow movements of the four limbs, epilepsy, and neurodevelopment affectations corresponding to a chronological age of 11 months. She showed an important decrease of the vision. The study showed a slight iron-deficiency anemia, immunoglobulin C3 and C4, blood chemistry at the normal limit. The magnetic resonance imaging showed important signs of cortical atrophy, stronger in the right temporal region. These are entities of scarce presentation and it is the only case with this diagnosis found in the province of Matanzas; that motivated this case presentation (AU).

Síndrome de Blue Rubber Bled Nevus. Presentación de un caso / Syndrome of Blue Rubber Bled Nevus. Case presentation

RESUMEN El síndrome de Blue Rubber Bled Nevus se caracteriza por múltiples malformaciones cutáneas, asociadas con malformaciones menores en órganos internos, (comúnmente afectan los órganos del sistema digestivo y el sistema nervioso central). A nivel mundial, se afirma que hay cerca de 1 a 2 millones de individuos afectados por este síndrome, y se cree que incide en 1 de cada 6000. En recién nacidos la incidencia se ha calculado en menos de 1 caso por 100 000 persona/año. Se presentó un caso de una paciente femenina de 8 años de edad, con antecedentes de hemangiomas múltiples de color azul violáceo, distribuidos por toda la superficie corporal, de diferentes diámetros. Algunos protruyen y depresibles al tacto. Presentaba ptosis palpebral y movimientos torpes de los cuatro miembros, epilepsia y afectaciones del neurodesarrollo el cual corresponde con una edad cronológica de 11 meses. Mostraba una disminución importante de la visión. Presencia de pie valgo a predominio derecho. Apareció anemia leve por déficit de hierro, inmunoglobulina C3 y C4, la química sanguínea al límite de lo normal. La resonancia magnética nuclear mostró importantes signos de atrofia cortical, más acentuados en región temporal derecha. Son entidades de muy escasa presentación y en la provincia de Matanzas es el único caso con este diagnóstico, por lo que constituyó el motivo para la presentación del caso.

ABSTRACT The syndrome of Blue Rubber Bled Nevus is characterized by multiple skin malformations, associated to minor malformations in internal organs (it commonly affects the organs of the digestive system and the central nervous system). It is affirmed that around 1 to 2 millions of individuals are affected by this syndrome worldwide, and it is believed that it has incidence on 1 of each 6000 persons. In newborns the incidence has been calculated in less than 1 case per 100 000 persons/year. The authors presented the case of a female patient, aged 8 years, with antecedents of purplish-blue multiple hemangiomas of different diameters, distributed all over the surface if the body. Several of them are protruding and depressible to the touch. She presented eyelid ptosis and slow movements of the four limbs, epilepsy, and neurodevelopment affectations corresponding to a chronological age of 11 months. She showed an important decrease of the vision. The study showed a slight iron-deficiency anemia, immunoglobulin C3 and C4, blood chemistry at the normal limit. The magnetic resonance imaging showed important signs of cortical atrophy, stronger in the right temporal region. These are entities of scarce presentation and it is the only case with this diagnosis found in the province of Matanzas; that motivated this case presentation.

Fatal Outcome from Brain Vascular Lesions in a Neonate with Blue Rubber Bleb Nevus Syndrome.

The most common cause of death in blue rubber bleb nevus syndrome is gastrointestinal bleeding. Here we present a case of central nervous system bleeding that resulted in death.

Melanoma tipo animal / Animal-type melanoma

El melanoma tipo animal o equino, es una variante infrecuente de melanoma con pronóstico incierto. Su nombre se debe a las similitudes histológicas con una neoplasia melanocítica presente en caballos tordillos ancianos. Clínicamente se presenta como una lesión nodular o una placa, con pigmentación intensa, pardo-azulada o negra. Histológicamente presenta principalmente una localización dérmica, observándose una proliferación profunda de células elongadas o redondeadas con moderada atipía ybaja tasa mitótica. Presentamos el caso de un paciente de sexo masculino de 31 años de edad, HIV +, con diagnóstico de melanoma tipo animal...

Blue rubber bleb nevus syndrome: case report.

The case of a patient with blue rubber bleb nevus syndrome who is infected by acquired immunodeficiency syndrome virus due to multiple blood transfusions is presented. This case shows that although it is a rare systemic disorder, blue rubber bleb nevus syndrome has to be considered in the differential diagnosis of chronic anemia or gastrointestinal bleeding. Patients should be investigated by endoscopy, which is the most reliable method for detecting these lesions. The patient underwent gastroscopy and enteroscopy via enterotomy with identification of all lesions. Minimal resection of the larger lesions and string-purse suture of the smaller ones involving all the layers of the intestine were performed. The string-purse suture of the lesions detected by enteroscopy proved to be an effective technique for handling these lesions, avoiding extensive intestinal resection and stopping the bleeding. Effective management of these patients demands aggressive treatment and should be initiated as soon as possible to avoid risks involved in blood transfusions, as occurred in this case.

Blue rubber bleb nevus syndrome: case report

The case of a patient with blue rubber bleb nevus syndrome who is infected by acquired immunodeficiency syndrome virus due to multiple blood transfusions is presented. This case shows that although it is a rare systemic disorder, blue rubber bleb nevus syndrome has to be considered in the differential diagnosis of chronic anemia or gastrointestinal bleeding. Patients should be investigated by endoscopy, which is the most reliable method for detecting these lesions. The patient underwent gastroscopy and enteroscopy via enterotomy with identification of all lesions. Minimal resection of the larger lesions and string-purse suture of the smaller ones involving all the layers of the intestine were performed. The string-purse suture of the lesions detected by enteroscopy proved to be an effective technique for handling these lesions, avoiding extensive intestinal resection and stopping the bleeding. Effective management of these patients demands aggressive treatment and should be initiated as soon as possible to avoid risks involved in blood transfusions, as occurred in this case

Blue rubber bleb nevus syndrome.

The blue rubber nevus syndrome consists of multiple venous malformations in the skin and gastrointestinal tract associated with intestinal hemorrhage and iron deficiency anemia. Other organs may be involved. The causes of this syndrome are unknown. Its most common presentation is in the form of sporadic cases, but dominant autosomal inheritance has been described. It is a condition that affects both sexes equally, and its occurrence is rare in the black race. We present a case of this syndrome diagnosed in a 11-year-old patient. He had severe anemia and a venous swelling on the trunk. Similar lesions were found in the stomach, bowel, and on his foot. We emphasize the main clinical aspects: intestine, eyes, nasopharynx, parotids, lungs, liver, spleen, heart, brain, pleura, peritoneum, pericardium, skeletal muscles, bladder, and penis lesions, systemic complications that may occur to these patients which are thrombosis and calcification, as well as consumptive coagulopathy and thrombocytopenia that may occur within the nevi.

Blue rubber bleb nevus syndrome

The blue rubber nevus syndrome consists of multiple venous malformations in the skin and gastrointestinal tract associated with intestinal hemorrhage and iron deficiency anemia. Other organs may be involved. The causes of this syndrome are unknown. Its most common presentation is in the form of sporadic cases, but dominant autosomal inheritance has been described. It is a condition that affects both sexes equally, and its occurrence is rare in the black race. We present a case of this syndrome diagnosed in a 11-year-old patient. He had severe anemia and a venous swelling on the trunk. Similar lesions were found in the stomach, bowel, and on his foot. We emphasize the main clinical aspects: intestine, eyes, nasopharynx, parotids, lungs, liver, spleen, heart, brain, pleura, peritoneum, pericardium, skeletal muscles, bladder, and penis lesions, systemic complications that may occur to these patients which are thrombosis and calcification, as well as consumptive coagulopathy and thrombocytopenia that may occur within the nevi.

Sindrome del nevo azul en tetina de goma / Blue rubber nevus syndrome

El sindrome del nevo azul en tetina de goma, Síndrome de Bean, o Blue rubber bleb nevus es una rara entidad consistente en la presencia de malformaciones vasculares múltiples( hemangioma cavernoso) en piel y órganos internos, principalmente aparato digestivo. Complicaciones como sangrado gastrointestinal, anemia i coagulop'atías, han sido documentadoas. Se comunica el caso de una niña de 12 años con "Sindrome del nevo azul en tetina de goma", que presento anemia y cuyo diagnóstico se realizó por biopsia y endoscopía digestiva(au)

Sindrome del nevo azul en tetina de goma / Blue rubber nevus syndrome

El sindrome del nevo azul en tetina de goma, Síndrome de Bean, o Blue rubber bleb nevus es una rara entidad consistente en la presencia de malformaciones vasculares múltiples( hemangioma cavernoso) en piel y órganos internos, principalmente aparato digestivo. Complicaciones como sangrado gastrointestinal, anemia i coagulopatías, han sido documentadoas. Se comunica el caso de una niña de 12 años con "Sindrome del nevo azul en tetina de goma", que presento anemia y cuyo diagnóstico se realizó por biopsia y endoscopía digestiva(au)

Tumores cutáneos dolorosos: segunda parte / Painful cutaneous tumors: second part

Se describe una serie clínico-patológica de tumores cutáneos habitualmente dolorosos y se revisan las características de cada uno de ellos, lipomatosis dolorosa, neurilemoma, neuroma, nevo azul en tetina de goma, sinovialoma, tumor de células granulosas y tumor glómico