Mastocitosis crónica. Presentación de un caso y revisión de la literatura

La mastocitosis cutánea crónica es una genodermatosis de etiología desconocida y que se encuentra dentro del grupo de enfermedades raras o poco frecuentes. Se caracterizan por el crecimiento y acumulación de causa desconocida de mastocitos en piel y otros órganos y se manifiesta como una urticaria pigmentosa. Se presenta el caso de un paciente de sexo masculino, con lesiones eritematopapulosas en región de las manos que fue extendiéndose a todo el cuerpo y cuero cabelludo que al desaparecer quedaban manchas hipercrómicas con signo de Darier positivo. Se confirmó el diagnóstico de urticaria pigmentosa mediante biopsia de la piel. Es una enfermedad rara con una baja incidencia, la forma cutánea tiene un pronóstico favorable, por lo que resulta de vital importancia conocer y diagnosticar de forma precoz esta entidad con un manejo multidisciplinario para prevenir las manifestaciones sistémicas que aparecen. Llevar un tratamiento sintomático permitirá una mejor calidad de vida en los infantes. Se presenta el caso porque al considerarse la mastocitosis una enfermedad rara la prevalencia e incidencia de estos casos es muy baja.

Chronic cutaneous mastocytosis is a genodermatosis of unknown etiology and that is within the group of rare or infrequent diseases. They are characterized by the growth and accumulation of mast cells in the skin and other organs of unknown cause and manifest as urticaria pigmentosa. The case of a male patient is presented, with erythematopapular lesions in the region of the hands that spread to the entire body and scalp that, when they disappeared, left hyperchromic spots with Darier's sign: positive. The diagnosis of urticaria pigmentosa was confirmed by skin biopsy. It is a rare disease with a low incidence, the cutaneous form has a favorable prognosis, so it is of vital importance to know and diagnose this entity early with a multidisciplinary management to prevent the systemic manifestations that appear. Taking a symptomatic treatment will allow a better quality of life in infants. The case is presented because mastocytosis is considered a rare disease, the prevalence and incidence of these cases is very low.

Mastocitoma felino. Reporte de caso / Feline mastocytoma. Case report

RESUMEN Los mastocitomas son tumores originarios de los mastocitos que usualmente afectan a los perros y los gatos. Pueden llegar a tener un comportamiento benigno, sin embargo, esto dependerá del grado de la neoplasia y su estadiaje. En felinos, clínicamente se han descrito dos patrones: visceral y cutáneo, de los cuales el cutáneo es el más frecuente, llegando a causar metástasis a órganos adyacentes e incluso afectar el bazo y/o intestino en estadios más diferenciados. Se presenta un caso de mastocitoma felino correspondiente a un ejemplar mestizo con cuadro clínico de inicio de más de dos meses de evolución, consistente en la aparición de una placa alopécica ulcerada y elevada en región interescapular, acompañada de prurito que presentó resolución espontánea. Mediante el curso clínico se imnunizó contra el virus de la rabia, posteriormente, se observó la aparición de una lesión nodular subcutánea de características inusuales de 2cm de diámetro. Acorde con el tiempo de evolución y el antecedente vacunal se decidió la realización de biopsia y resección quirúrgica. El reporte de la biopsia confirmó diagnóstico de mastocitoma grado histológico 3 de Patnaik, teniendo en cuenta las características histológicas, estructurales y su comportamiento clínico. Se realizó seguimiento del caso pasados ocho meses, donde se evidenció mejoría del cuadro clínico, sin aparición de nueva masa sugestiva de neoplasia, sin hallazgos de metástasis a otras estructuras, con cicatrización exitosa de herida quirúrgica y evolución satisfactoria.

ABSTRACT Mast cells are tumors originating from mast cells which usually affect cats and dogs. They may have benign behavior, however, this will depend on the degree of the neo-plasm and its staging. In cats, two patterns have been described clinically: visceral and cutaneous, of which the cutaneous form is the most frequent, reaching metastasis to adjacent organs and even affecting the spleen and / or intestine in more differentiated stages. A case of a mastocytoma is presented, a feline corresponding to a mongrel specimen with a clinical picture of onset of more than two months of evolution, consisting of the appearance of an allopecal plaque, ulcerated and elevated in the interscapular region, accompanied by pruritus that presented spontaneous resolution. Through the clinical course, he was immunized against the rabies virus, later the appearance of a nodular lesion of unusual subcutaneous characteristics, 2 cm in diameter, was observed. According to the evolution time and the vaccination history, it was decided to perform a biopsy and surgical resection. The biopsy report confirmed the diagnosis of Patnaik's histological grade 3 mastocytoma, taking into account the histological and structural characteristics and its clinical behavior. The case was followed up after eight months, where an improvement in the clinical picture was evident, without the appearance of a new mass suggestive of neoplasia, without metastatic findings to other structures, with successful healing of the surgical wound and satisfactory evolution.

Mastocitoma solitario ampollar / Solitary Blister Mastocytoma

La mastocitosis comprende un conjunto de desórdenes con expansión anormal y acumulación de mastocitos en diversos órganos incluida la piel, en la cual se describen distintas formas clínicas de presentación. El mastocitoma cutáneo solitario constituye el 10-15% de todas las mastocitosis. Presentamos el caso de un niño con mastocitoma cutáneo solitario ampollar, un cuadro infrecuente cuyo diagnóstico oportuno constituye un reto para pediatras y dermatólogos (AU)

Mastocytosis comprises a set of disorders with abnormal expansion and accumulation of mast cells in various organs including the skin, wich describes different form of clinical presentation. The solitary cutaneous mastocytoma constitutes 10-15 % of all mastocytosis. We present the case of a child with solitary blistering mastocytoma, an infrequent condition whose, timely diagnosis constitutes a challenge for pediatricians and dermatologists (AU)

Mastocitosis solitaria en pediatría. Presentación de un caso y revisión de la literatura / Solitary Mastocytoma in Pediatrics. Case Report and Literature Review

RESUMEN El mastocitoma es una genodermatosis de etiología desconocida, por lo que se incluye en el grupo de enfermedades raras o pocos frecuentes; aparece generalmente en la infancia o al momento de nacer. Se caracteriza por acumulación local o sistémica de células cebadas, lo que origina la aparición de lesiones cutáneas persistentes, pigmentadas y muy pruriginosas con compromiso exclusivo de la piel, se presenta generalmente como una lesión única en el cuello, el tronco, los brazos y las piernas. Las características típicas son las de un nódulo color marrón rojizo o amarillento, de consistencia gomosa, la superficie con aspecto de piel de naranja y signo de Darier. Se presenta el caso de un lactante de 4 meses de edad con un diagnóstico de mastocitoma solitario con signo de Darier. Se realiza este reporte por la baja frecuencia de esta patología en el Hospital Pediátrico Universitario Paquito González Cueto de Cienfuegos. Actualmente el paciente se mantiene con seguimiento por consulta de dermatología, con tratamiento antihistamínico y estabilizador de mastocitos.

ABSTRACT The mastocytoma is a genodermatosis of unknown etiology, so it is included in the group of rare or uncommon diseases; it usually appears in childhood or at birth. It is characterized by local or systemic accumulation of primed cells, which causes the appearance of persistent, pigmented and very itchy skin lesions with exclusive skin involvement, usually presents as a single lesion in the neck, trunk, arms and legs. Typical features are that of a reddish-brown or yellowish brown nodule, of gummy consistency, the surface looks like orange peel, and Darier's sign. The case of a 4-month-old infant with a diagnosis of solitary mastocytoma with Darier's sign is presented. This report is made due to the low frequency of this disease at the Paquito González Cueto de Cienfuegos University Pediatric Hospital. Currently the patient is monitored by dermatology specialists, with antihistamine treatment and mast cell stabilizer.

Solitary mastocytoma presenting at birth

Mastocytosis is a rare disease which occurs in both children and adults, and it can manifest as a solitary or multiple skin lesions. Both can cause cutaneous or systemic symptoms. Because of the heterogeneity of clinical presentation of mastocytosis and its rare prevalence, it can be hard to suspect the mastocytosis at the first time. Most solitary mastocytomas are about 1–5 cm in diameter and have features of brownish-yellow, minimally elevated plaques with a smooth shiny surface. This article presents a case of solitary mastocytoma which occurred in neonate and that we treated through surgical excision. In histopathological examination, it consisted of c-kit-positive mast cells. Although pediatric cutaneous mastocytosis might regress spontaneously, clinicians should keep in mind that it could be associated with systemic mastocytosis which involves hematopoietic system.

Mastocitosis cutánea: reporte de un caso / Cutaneous mastocytosis: A case report

Introducción La mastocitosis representa un grupo de enfermedades caracterizadas por una acumulación excesiva de mastocitos en uno o múltiples tejidos. Puede limitarse a la piel o tener un compromiso sistémico, siendo de baja prevalencia y pronóstico benigno en la infancia. Objetivo Reportar un caso de urticaria pigmentosa como subtipo de mastocitosis cutánea y hacer una revisión bibliográfica enfocada en los hallazgos clínicos, el diagnóstico y el manejo inicial básico. Caso clínico Lactante de 6 meses de edad con múltiples máculas y pápulas de color café claro localizadas en el tronco, los brazos y las piernas, cuadro compatible con una urticaria pigmentosa, confirmada mediante biopsia. Se solicitaron exámenes para descartar compromiso sistémico. La paciente fue tratada con medidas generales, educación y antihistamínicos, con excelente evolución. Conclusiones La mastocitosis cutánea es una enfermedad poco común, de buen pronóstico. En la infancia generalmente bastan las medidas generales y educación para obtener resultados favorables. La terapia farmacológica de primera línea son los antagonistas H1.

Introduction Mastocytosis represents a group of diseases characterised by an excesive accumulation of mastocytes in one or multiple tissues. It can affect only the skin, or have a systemic involvement. It has a low prevalence, and the prognosis is benign in children. Objective To report a case of urticaria pigmentosa as a subtype of cutaneous mastocytosis, and present a literature review focused on clinical findings, diagnosis and initial basic management. Clinical case A child of six months of age presenting with multiple blemishes and light brown papules located on the trunk, arms and legs. The symptoms were compatible with urticaria pigmentosa, and was confirmed by biopsy. Tests to rule out systemic involvement were requested. The patient was treated with general measures, education, and antihistamines, with favourable results. Conclusions Cutaneous mastocytosis is a rare disease with a good prognosis. In childhood general measures and education are usually enough to obtain favourable results. Histamine H1 antagonists are the first line drug treatment.

Mastocitoma cutáneo solitario: A propósito de un caso / Solitary cutaneous mastocytosis: A case report

Se presenta una niña de 16 meses de edad, con una lesión de catorce meses de evolución que consistía en una única placa eritematosa, en forma de L, bien delimitada, localizada en la parte superior de la espalda. El signo de Darier fue positivo. La histopatología mostró edema intersticial y en la tinción con Giemsa, se observaron mastocitos. Se realizó el diagnóstico de un mastocitoma solitario. El mastocitoma solitario es una lesión tumoral benigna, que representa aproximadamente el 15% de la mastocitosis cutánea.

We report a 16 month years old female, with a 14 months history of a single erythematous, L shaped well-delimited plaque located on her upper back. Darier sign was positive. Histopathology revealed interstitial edema. The Giemsa staining showed mast cells. The diagnosis of a solitary mastocytoma was performed. Solitary mastocytoma is a benign tumoral lesion, that represents approximately 15% of cutaneous mastocytosis.

Clinical and prognostic analysis of 183 cases of pediatric cutaneous mastocytosis / 中华皮肤科杂志

Objective To characterize clinical features and prognosis of pediatric cutaneous mastocytosis. Methods Clinical data on 183 cases of pediatric cutaneous mastocytosis were collected and retrospectively analyzed. Some patients were followed up. Results Of the 183 patients, 136 (74.3%)had urticaria pigmentosa, 43 (23.5%) mastocytoma, 4 (2.2%)diffuse mastocytosis. The first attack of mastocytosis occurred at birth in 21 (48.8%)patients with mastocytoma and 35(25.7%)patients with urticaria pigmentosa, within 6 months after birth in 17(39.5%)patients with mastocytoma and 78(57.3%)patients with urticaria pigmentosa, and within 2 years after birth in 179(97.8%)out of the 183 patients. Of 33 patients with detailed description of symptoms, 10 had concomitant symptoms, which were flushing in 9 patients. Forty-five patients were followed up for 3 - 6 years (average, 4 years). The follow-up showed that skin lesions completely regressed in 1 patient with urticaria pigmentosa at 11 years of age, partially regressed in 18 patients. Lesions regressed completely at 8 years of age in 1 patient with mastocytoma, and subsided within 1 year after skin biopsy in 7 patients. Oral antihistamines could control the symptoms of mastocytosis, such as flushing, whealing and blistering, and oral glucocorticoids could effectively control the recurrence of generalized blisters and bullae in patients with diffuse mastocytosis. Conclusions Urticaria pigmentosa appears to be the most common type of cutaneous mastocytosis in children, followed by mastocytoma. Mastocytoma occurs most frequently at birth, while urticaria pigmentosa within 6 months after birth. Oral antihistamines may control inflammatory mediator-related symptoms. Serious diffuse mastocytosis may be controlled by systemic glucocorticoids.

A Case of Solitary Bullous Mastocytoma Occurring at a Site of Spinal Tap / 대한피부과학회지

Mastocytosis is characterized by an accumulation of mast cells in various organs, most frequently in the skin. A solitary mastocytoma is a clinical variant of cutaneous mastocytosis. It is defined as a localized collection of mast cells in the skin without evidence of extracutaneous organ involvement. Here we report on a 2-year-old female patient presenting with Solitary erythematous bulla on her lower back. The patient had a history of spinal tap on the lower back for evaluation of meningitis at 5 months of age, which resulted in trauma at the site. Histopathology showed mast cells infiltrating the papillary and reticular dermis and metachromatic purple cytoplasmic granules seen with Giemsa staining. As a result, the patient was diagnosed with a solitary bullous mastocytoma and administered antihistamine. The patient showed complete remission at 3 months. Herein, we report a rare case of solitary bullous mastocytoma occurring at a trauma site.

Mastocitoma solitario: a propósito de un caso / Solitary mastocytoma: a propos of a case

El mastocitoma solitario es la segunda en frecuencia, de las variantes de las mastocitosis, caracterizada por una proliferación de los mastocitos tisulares que afecta únicamente a la piel, comúnmente congénita o de aparición en las primeras semanas de vida, con etiología aún desconocida y buen pronóstico, ya que tiene una resolución espontánea en la adolescencia. Presentamos el caso de un paciente quien desde su nacimiento, presentaba una placa en antebrazo derecho, con signo de Darier negativo y asintomatomática. Con los datos clínicos y los antecedentes personales se realiza biopsia de piel, mediante la que confirmamos el diagnóstico de mastocitoma solitario.

Solitary mastocytoma is the second most frequent disease within mastocytosis. It is characterized by a hyperplasia of the mastocitos tissue that affects only the skin. It is usually congenital though it can also appear in the first weeks of life, with unknown etiology; it has a good prognosis due to its spontaneous resolution in the adolescence. This paper presents case of a patient who, from birth, has had some plaque in his right forearm, with negative and asymptomatic Darier's sign. The skin biopsy has been performed with clinical data and personal history, thus confirming the diagnosis of solitary mastocytoma.

Behavior of the P1.HTR mastocytoma cell line implanted in the chorioallantoic membrane of chick embryos

The P1.HTR cell line includes highly transfectable cells derived from P815 mastocytoma cells originating from mouse breast tissue. Despite its widespread use in immunogenic studies, no data are available about the behavior of P1.HTR cells in the chick embryo chorioallantoic membrane model. The objective of the present investigation was to study the effects of P1.HTR cells implanted on the chorioallantoic membrane of chick embryos. We inoculated P1.HTR cells into the previously prepared chick embryo chorioallantoic membrane and observed the early and late effects of these cells by stereomicroscopy, histochemistry and immunohistochemistry. A highly angiotropic and angiogenic effect occurred early after inoculation and a tumorigenic potential with the development of mastocytoma keeping well mast cells immunophenotype was detected later during the development. The P1.HTR mastocytoma cell line is a good tool for the development of the chick embryo chorioallantoic membrane mastocytoma model and also for other studies concerning the involvement of blood vessels. The chick embryo chorioallantoic membrane model of mastocytoma retains the mast cell immunophenotype under experimental conditions and could be used as an experimental tool for in vivo preliminary testing of antitumor and antivascular drugs.

Mastocitoma cutáneo solitario: presentación de un caso / Solitary cutaneous mastocytoma: a case report

Se presenta el caso de un lactante menor con tumoración cutánea benigna, localizada en el área submandibular izquierda de la cabeza, característico de un mastocitoma solitario. Su reconocimiento clínico precoz y la instauración terapéutica temprana aceleraron el tiempo de resolución, casi siempre espontánea.

We present a case of an infant with benign cutaneous tumor, located in the left submandibular aspect of the head consistent with solitary mastocytoma. Its early clinical recognition and early treatment onset accelerates time to resolution almost always spontaneous.

Mastocitoma solitario: presentación de un caso / Solitary mastocytoma: report of a case

Se presenta el caso de un lactante menor con tumoración cutánea benigna localizada en el aspecto submandibular izquierdo de la cabeza compatible con mastocitoma cutáneo solitario. Esta rara lesión tumoral es parte del grupo heterogéneo de las mastocitosis, conjunto de enfermedades que resulta de la infiltración anómala de mastocitos en diferentes tejidos corporales, afectando particularmente la piel.La variedad más común de mastocitosis en niños es la urticaria pigmentosa, seguida del mastocitoma cutáneo que contabiliza entre el 10 y 15% de todos los casos. Suele situarse en tronco o extremidades, aunque como en este paciente puede localizarse en cabeza uotros sitios corporales. Es de aparición precoz durante la primera infancia o alguna vez congénito; por lo regular no hay participación de órganos internos o situaciones potencialmente letales. Su curso es crónico con remisiones parciales y exacerbaciones sintomáticasmolestas como fenómenos de urticación y desarrollo de ampollas frente a estímulos externos o internos que preocupan a los familiares cercanos. Su reconocimiento clínico precoz y la pronta instauración terapéutica con esteroides locales, antihistamínicos orales u otras medidas pertinentes, aceleran el tiempo de resolución casi siempre espontánea hacia los 2 años de vida; es importante la debida explicación a los padres

This is a report the case of an infant with benign cutaneous tumor located in the left submandibular aspect of the head support solitary cutaneous mastocytoma. This rare tumor lesion is part of the heterogeneous group of mastocytosis, group of diseases resulting from abnormal infiltration of mast cells in various body tissues, particularly affecting the skin. The most common variety of mastocytosis inchildren is urticaria pigmentosa, followed by cutaneous mastocytoma counted between 10 and 15% of all cases. Usually located on the trunk or extremities, although as in this patient may be located in the head or other body sites. Is early onset in infancy or congenital ever, usually no involvement of internal organs or life-threatening situations. Its course is chronic symptomatic partial remissions and exacerbations Urtication annoying as phenomena and development of blisters against external or internal stimuli which trouble close relatives. Its early clinical recognition and prompt establishment local steroid therapy, oral antihistamines or other relevant measures speed time to spontaneous resolution usually 2 years of life, is important due explanation to parents.

Two Cases of Infant-onset Cutaneous Mastocytosis / 임상소아혈액종양

Mastocytosis is a rare disease in infants and children that characterized by a pathologic increase and accumulation of mast cells in one or more organs. Cutaneous mastocytosis is a typical presentation of pediatric-onset mastocytosis and often presents classical symptoms and signs related with mast cell mediator including pruritus, flushing, abdominal pain and Darier's sign. We present our experience with two rare cases of cutaneous mastocytosis, a 3-month-old boy and a 4-month-old boy. The former had a recurrent single brownish plaque with yellowish bullae in right forearm without any other symptom or sign. The latter had multiple brownish plaques in his trunk and extremities with intermittent diarrhea and Darier's sign. In each, a punch biopsy confirmed the diagnosis of solitary mastocytoma and urticaria pigmentosa. After 2weeks of anti-histamine and leukotriene antagonist therapy for symptom relief, both infants had no remnant skin lesion or symptoms.

Two Cases of Infant-onset Cutaneous Mastocytosis / 임상소아혈액종양

Mastocytosis is a rare disease in infants and children that characterized by a pathologic increase and accumulation of mast cells in one or more organs. Cutaneous mastocytosis is a typical presentation of pediatric-onset mastocytosis and often presents classical symptoms and signs related with mast cell mediator including pruritus, flushing, abdominal pain and Darier's sign. We present our experience with two rare cases of cutaneous mastocytosis, a 3-month-old boy and a 4-month-old boy. The former had a recurrent single brownish plaque with yellowish bullae in right forearm without any other symptom or sign. The latter had multiple brownish plaques in his trunk and extremities with intermittent diarrhea and Darier's sign. In each, a punch biopsy confirmed the diagnosis of solitary mastocytoma and urticaria pigmentosa. After 2weeks of anti-histamine and leukotriene antagonist therapy for symptom relief, both infants had no remnant skin lesion or symptoms.

Mastocitosis cutánea: revisión de 10 años de experiencia en el Servicio de Dermatología del Hospital General de Niños Pedro de Elizalde / Cutaneous mastocytosis: 10-years experience review at the Dermatology Department, Hospital General de Niños Pedro de Elizalde

Introducción. La mastocitosis representa un espectro de trastornos clínicos con un fenotipo común correspondiente a una hiperplasia de los mastocitos tisulares. Objetivo. Presentar la experiencia del Servicio de Dermatología del Hospital General de Niños Pedro de Elizalde en mastocitosis cutánea en los últimos 10 años. Diseño. Estudio retrospectivo y descriptivo, de corte transversal. Material y método. Se revisaron los archivos histológicos correspondientes al período que va de marzo de 1999 a marzo de 2099 del Servicio de Dermatología del Hospital General de Niños Pedro de Elizalde. Se consideraron como variables de estudio la edad al momento de la consulta, sexo, presencia de antecedentes personales y familiares, tipo clínico, localización, presencia de síntomas al examen físico, así como hallazos patológicos en los estudios complementarios. Los resultados se expresaron en promedios y porcentajes. Resultados. Presentamos 49 casos con diagnóstico clínico e histológico de mastocitosis cutánea; 33 pacientes (67,3%) de sexo masculino y 16 (32,7%) de sexo femenino. En cuanto al tipo clínico de mastocitosis, se encontraron 36 casos de urticaria pigmentosa (73,4%), 6 casos de mastocitoma solitario (12,2%), 5 casos de telangiectasia macularis eruptiva perstans (10,2%) y 2 casos donde coexistía mastocitoma solitario y urticaria pigmentosa (4%). No se encontraron casos de mastocitosis cutánea difusa. Las lesiones se distribuyen en tronco 25 casos (51%), en tronco y miembros 17 casos (34,6%), en cabeza, tronco y miembros 3 casos (6,1%), en miembros 3 casos (6,1%), en cabeza y tronco un caso (2,04%). Conclusión. En nuestra casuística, la manifestación clínica más frecuente fue la urticaria pigmentosa, similar a lo señalado en la bibliografía. Encontramos, a diferencia de lo publicado en la literatura, un predominio de sexo masculino y un alto porcentaje (10,2%) de pacientes pediátricos con la forma de telangiectasia macularis eruptiva perstans.

IntroductionMastocytosis represents a spectrum of clinical disorders with a common phenotype correspondingto hyperplasia of mast cells on tissues.ObjectiveTo present the experience of the Department of Dermatology, Hospital General de Niños Pedro deElizalde in cutaneous mastocytosis during the past 10 years.DesignRetrospective and descriptive, cross sectional study.Material and methodsHistological records were reviewed for the period March 1999-March 2009 at the Department ofDermatology, Hospital General de Niños Pedro de Elizalde.The variables considered were: age at time of consultation, gender, presence of personal andfamily history, clinical type, location, presence of symptoms by physical examination andabnormal findings on complementary studies.The results were expressed as averages and percentages.ResultsForty nine patients with clinical and histological diagnosis of cutaneous mastocytosis arepresented, 33 of them were male (67.3%) and 16 were female (32.7%).As regards to the clinical type of mastocytosis, 36 patients presented urticaria pigmentosa(73.4%), 6 had solitary mastocytoma (12.2%), 5 presented telagiectasia macularis eruptivaperstans (10.2%) and 2 patients presented solitary mastocytoma combined with urticariapigmentosa (4%). Diffuse cutaneous mastocytosis was not found.Twenty five patients had lesions distributed on the trunk (51%), trunk and limbs on 17 (34.6%),head, trunk and limbs 3 patients (6.1%), on limbs only 3 patients (6.1%), while head and trunktogether were affected on 1 patient (2.04%).ConclusionIn our caseload, the most frequent clinical manifestation was urticaria pigmentosa, similar tofindings in the literature.As opposed to published data we found predominance in males, and a high incidence (10.2%)of pediatric patients with telangiectasia macularis eruptiva perstans.

Combined Mastocytoma-hemangioma in a Patient with Urticaria Pigmentosa

A patient with long-standing urticaria pigmentosa presented with a pea-sized reddish to purplish papule on the posterior part of the right ear. Histopathologic examination revealed numerous dilated vascular structures in the upper dermis and mast cell infiltrations throughout the whole dermis, consistent with combined mastocytoma-hemangioma. The mast cells were strongly positive with Giemsa stain.

Mastocitoma solitario: Presentación de un caso en el adulto / Solitary mastocytoma: Presentation in an adult

El objetivo de esta publicación es mostrar un caso de mastocitoma solitario en la vejez, debido a la rareza de su aparición en tal etapa. Sus diagnósticos diferenciales y la involución total de la lesión con el uso de apósitos hidrocoloides, son comentados.

We present a case of a solitary mastocytoma in a man 86 years old who developped the lesion on the inferior lip. The interest of the case is the rarity of the presentation in an adult, which presents others differential diagnostics, and the success of the treatment with hidrocolloid dressings.

Mastocitoma solitario congénito: reporte de un caso / Congenital solitary mast cell tumor: case report

El mastocitoma solitario es una variedad de mastocitosis cutánea que se da desde el nacimiento o durante los primeros seis meses de edad. Se presenta como una lesión única macular, placa o nódulo eritematoso, amarillento o hiperpigmentado, que a la fricción es pruriginosa y se torna más eritematosa, edematosa o ampollar; pero también puede presentar signos extralesionales. El curso natural del cuadro es de involución espontánea en la mayoría de casos, pero dependiendo de los signos y síntomas asociados, se indica antihistamínicos, corticoides tópicos o intralesionales, e incluso excisión quirúrgica (AU)

Solitary mastocytoma is a variety of cutaneous mastocytosis, which usually appears in newborns or during the first six months of age. It presents as a single macule, plaque or nodule that can be erythematous, yellowish or hyperpigmented; with friction it becomes pruritic, erythematous, oedematous or bullous, but may also have extralesional signs. The natural course in most cases is spontaneous involution. Depending on the manifestations, antihistamines, topical or intralesional corticosteroids and even surgical excision can be indicated (AU)