Anaphylaxis and systemic mastocytosis caused by Solenopsis invicta stings / Anafilaxia e mastocitose sistêmica ocasionada pela Solenopsis invicta

Indolent systemic mastocytosis is a rare disease characterized by an increased number of mast cells in the bone marrow and other tissues, such as the liver, spleen, lymph nodes, and skin. Patients with indolent systemic mastocytosis and high serum tryptase levels are at risk for Hymenoptera venom-induced anaphylaxis. Hymenoptera venom immunotherapy in patients with specific IgE is safe and effective. While some patients can receive ultra-rush venom immunotherapy with minimal side effects, omalizumab effectively protects against anaphylaxis during the build-up phase.

A mastocitose sistêmica indolente é uma doença rara caracterizada por um número aumentado de mastócitos na medula óssea e em outros tecidos, como fígado, baço, linfonodos e pele. Pacientes com mastocitose sistêmica indolente e altos níveis séricos de triptase correm risco de anafilaxia induzida pelo veneno dos Hymenoptera. A imunoterapia com veneno de himenópteros em pacientes com IgE específica é segura e eficaz. Embora alguns pacientes possam receber imunoterapia com veneno ultrarrápido com efeitos colaterais mínimos, o omalizumabe protegeu efetivamente contra a anafilaxia durante a fase de acúmulo.

Study of Mast Cells in Non Neoplastic Skin Conditions

Introduction: Mast cells are always been a fascinating field of research workers working with inflammatory processes, Hypersensitive disorders, and hosts defence mechanisms. Wide variation in mast cell counts and blood density is noticed in different sections of the skin in same biopsy sample. Aim and objectives: To assess the pattern of mast cell arrangement in various non- neoplastic skin lesions and compared with normal skin biopsies, to analyze the pattern of mast cell distribution in various non-neoplastic lesions of the skin, to compare with quantum of mast cells in various nonneoplastic lesions. Materials and methods: A study was conducted for 10 years from March 2006 to February 2016, which included all non-neoplastic skin conditions were subjected for mast cell count and distribution in the Department of Pathology at Gandhi Medical College/ Hospital with collaboration with Dermatology Department. The biopsy specimens were processed and stained with routine H&E and special stain for mast cells Toluidine Blue. The mast cells counted with the help of ocular micrometer under the microscope. Results: About 1000 non-neoplastic lesions grouped into 11 categories and mast cell count was done. Irrespective of the category, Urticaria pigmentosa showed highest mast cell count (144/sqmm). Nevus comedonicus showed lowest count (13/sqmm). Conclusion: The role of mast cells in health and disease is significant and distinct alterations of mast cell are present in various non-neoplastic conditions.

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.

Urticaria Pigmentosa de presentación atípica en la infancia: reporte de un caso clínico / Pigment urticaria of atypical presentation in childhood: clinical case report

La Urticaria Pigmentosa es el tipo más frecuente de mastocitosis cutánea (MC) de inicio en la edad pediátrica, representando el 75 por ciento de las MC. Se presenta con máculas y/o pápulas de color amarillo a café-rojizo, usualmente con signo de Darier positivo y prurito. El 88 por ciento presenta compromiso de tronco. En este artículo compartimos el caso de Urticaria Pigmentosa de presentación atípica: predominio de lesiones en cara y sin compromiso de tronco.(AU)

Urticaria Pigmentosa is the most common form of cutaneous mastocytosis (CM) starting in childhood and accounts for 75 percent of all the CM cases. It presents with yellow to brown-reddish macules or papules, usually with positive Darier sign and itching. Trunk involvement is present in 88 percent of patients. In this article we describe a case of Urticaria Pigmentosa with an atypical presentation: facial predominance without trunk involvement. (AU)

Mastocitosis: urticaria pigmentosa / Mastocytosis: urticaria pigmentosa

La mastocitosis es una enfermedad rara. Se caracteriza por una proliferación benigna o en raras ocasiones maligna de mastocitos en los tejidos. Puede producir alteraciones tanto sistémicas como cutáneas. En las edades pediátricas la forma cutánea más frecuente es la urticaria pigmentosa con muy buen pronóstico en la niñez; generalmente sin manifestaciones sistémicas y se resuelve sin secuelas en la pubertad en un 50% de los casos. Sin embargo, no es bien conocida por los médicos por ser poco común en la infancia. Se presenta un caso clínico que a los dos meses de vida comenzó a tener en la piel lesiones máculo-papulosas de color rosado parduzco, simétricas de distribución centrípeta; diseminadas con tendencia a la agrupación y predilección por el tronco y extremidades. Fue diagnosticado como una urticaria pigmentosa. Ha mantenido una evolución satisfactoria sin repercusión sistémica ni manifestaciones extracutáneas con un seguimiento clínico por pediatría cada seis meses y anual por el especialista en dermatología, para observar su evolución hasta los 5 años de edad, sin necesidad de tratamiento citorreductor; solo con tratamiento sintomático en ocasiones.

Mastocytosis is a rare disease. It is characterized by benign or on rare occasions malignant proliferation of mast cells in tissues. It can produce both systemic and cutaneous changes. In pediatric patients the most common cutaneous form is urticaria pigmentosa with very good prognosis in childhood; usually without systemic manifestations and resolved without sequelae at puberty in 50% of cases. However, it is not well known by doctors as being rare in childhood. The case of a two month-old male patient presented maculo-papulous skin lesions, with brownish symmetrical pink centripetal distribution lesions; scattered and prone to aggregation, presenting a predilection for the trunk and extremities, the diagnosis was: urticaria pigmentosa. The patient has maintained a satisfactory evolution without systemic repercussions or extracutaneous manifestations, he maintains clinical monitoring every six months and annually in dermatology service, in order to observe the natural history of the disease until the age of 5, without cytoreductive therapy; only with symptomatic treatment occasionally.

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.

Urticaria pigmentosa: a propósito de un caso de inicio en la edad adulta / Urticaria pigmentosa: a case report of adulthood development

Se presenta el caso de una paciente femenina de 41 años de edad, con dermatosis diseminada a tronco y porción proximal de extremidades, caracterizada por una erupción máculo-papular muy pruriginosa de curso crónico, con exacerbaciones lesionales por estímulos externos físicos, térmicos y remisiones parciales, tratada con antihistamínicos orales de manera irregular. Se hizo el diagnóstico clínico de urticaria pigmentosa por el aspecto morfológico, corroborado a través del estudio anátomo-patológico. La urticaria pigmentosa es la variante más común del amplio espectro de las mastocitosis, propia de la niñez y excepcional en adultos, sin compromiso orgánico interno. El interés de esta presentación, se fundamenta en la escasa prevalencia documentada de esta patología en la adultez, sin evidenciar participación infiltrativa del tejido óseo-medular en el control evolutivo.

We report the case of a female patient of 41 years-old, with disseminated dermatosis on trunk and proximal portion of the extremities, characterized by highly itching maculopapular rash of chronic course with exacerbations, caused by external physical and thermal stimuli with partial remissions treated with oral antihistamines irregularly. The clinical diagnosis of urticaria pigmentosa was made and confirmed by pathologic examination. Urticaria pigmentosa is the most common variant of the wide spectrum of mastocytosis, a disease occasionally observed in children and rarely in adults, without internal organ involvement. The interest of this presentation is based on the low reported prevalence of this disease in adulthood without evidence of infiltrative involvement of the medullar and bone tissue in regular monitoring of control.

Dermatoscopic findings of urticaria pigmentosa / Achados dermatoscopicos na urticaria pigmentosa

Mastocytosis is a rare disease characterized by proliferation and accumulation of mast cells in various organs. The maculopapular cutaneus mastocytosis is divided into three subtypes: papular/plaque variant, urticaria pigmentosa and eruptive macular telangiectasia perstans. Dermoscopic may help to better characterize the different forms of cutaneus mastocytosis. We report a 55 year-old female with urticaria pigmentosa and its dermoscopy.

Mastocitose é uma doença rara caracterizada pela proliferação e acúmulo de mastócitos em vários órgãos. A mastocitose cutanea maculopapular divide-se em três subtipos: variante máculo-papular, urticária pigmentosa e telangiectasia macular eruptiva perstans. A dermatoscopia pode ajudar a caracterizar melhor as diferentes formas de mastocitose cutânea. Relatamos um caso de paciente feminina de 55 anos com urticária pigmentosa e sua dermatoscopia.

Mastocitosis. Presentación de un caso / Mastocytosis. A case report

La mastocitosis o mastocitoma es una genodermatosis poco frecuente, de etiología desconocida, incluida en el grupo de "enfermedades raras", que aparece en la infancia generalmente. Se relaciona con procesos de hipersensibilidad, enfermedades crónicas o neoplásicas. Se caracteriza por acumulación local o sistémica de células cebadas, dando lesiones cutáneas pruriginosas, persistentes y pigmentadas de diferentes tamaños. Se presenta un adolescente de 17 años, masculino, con lesiones cutáneas de seis años de evolución, del área de salud del Pol. "Dr. Isidro de Armas", Municipio Playa, La Habana, cuyo síntoma predominante era el prurito y enrojecimiento de cara. Luego del estudio realizado, se diagnostica una mastocitosis, variedad urticaria pigmentosa. Se constatan malformaciones óseas y tendencia a variante sistémica. Se trata para aliviar los síntomas, e interconsulta con otras especialidades para mejorar su calidad de vida. Se demuestra la importancia del método clínico para llegar al diagnóstico positivo de una enfermedad poco frecuente y de pronóstico reservado, en la Atención Primaria.

Mastocytosis or mast cell tumor is a rare genodermatosis of unknown etiology, belonging to the group of "Rare Diseases" that appear generally during childhood. It is related to hypersensitivity processes, chronic diseases or neoplasms. It is characterized by local accumulation or systemic mast cells (mastocytes), causing cutaneous pruritogenic, persistent and different-sized pigmented lesions. A 17- year-old male adolescent presented cutaneous six-year progression lesions, the patient belonged to the health area of "Dr. Isidro de Armas" outpatient clinic, Playa municipality, Havana. The prevailing symptoms were pruritus and redness of face. After the study carried out, mastocytosis was diagnosed, urticaria pigmentosa variety; observing osseous malformations and a systemic variant set. Symptoms were treated to be relieved, along with referrals to other medical specialties to improve his quality of life. The importance of clinical method to establish a positive diagnosis of a rare disease and its uncertain prognosis was demonstrated in Primary Health Care.

Urticária pigmentosa: mastocitose cutânea na infância / Urticaria pigmentosa: cutaneous mastocytosis in a child

A mastocitose é uma doença rara caracterizada pela proliferação e subsequente acúmulo de mastócitos na pele e/ou órgãos internos. Esta patologia pode ser dividida em cutânea e sistêmica. A cutânea é mais frequente na infância; o infiltrado de mastócitos é limitado à pele, geralmente tem uma evolução benigna e regride espontaneamente na adolescência. A urticária pigmentosa é a apresentação cutânea mais comum. São máculas, pápulas, nódulos ou placas, de coloração vermelho-acastanhada, com predileção pelo tronco. O sinal de Darier ocorre em 90% dos pacientes. Este trabalho tem como objetivo relatar um caso de mastocitose cutânea na infância, no qual o quadro clínico e a biópsia de pele foram suficientes para o diagnóstico. Em pacientes com mastocitose, é importante enfatizar a necessidade do acompanhamento periódico com realização de exames laboratoriais e de imagem, conforme a sintomatologia apresentada pelo paciente, e informar a família sobre a doença e acerca da maior predisposição à anafilaxia.

Mastocytosis is a rare disease characterized by proliferation and subsequent accumulation ofmast cells in the skin and/or internal organs. Presentation can be cutaneous or systemic. Thecutaneous form is more common in childhood; mast cell infiltration is limited to the skin, clinicalcourse is usually benign, and the disease regresses spontaneously in adolescence. Urticariapigmentosa is the most common cutaneous presentation. It includes macules, papules, nodules,or plaques, brownish red in color, with a predilection for the trunk. Darier’s sign is present in 90%of the patients. The present study reports on a case of cutaneous mastocytosis in childhood inwhich clinical evaluation and skin biopsy were sufficient to establish diagnosis. In patients withmastocytosis, regular monitoring with laboratory tests and imaging exams is extremely important,according to the symptoms presented by the patient. Likewise, the need to inform the familyabout the characteristics of the disease and the higher risk of anaphylaxis is underscored.

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.

Urticaria pigmentosa en el adulto / Urticaria pigmentosa in adult

La urticaria pigmentosa es una de las formas más comunes de las mastocitosis, las cuales corresponden a una serie de procesos caracterizados por un aumento anormal de los mastocitos en diversos órganos y tejidos corporales. En la urticaria pigmentosa la manifestación es exclusiva de la piel, caracterizada por la presencia de lesiones maculopapulares de 2.5 a 5 mm de diámetro, de un color que puede oscilar entre rojo y café, distribuyéndose generalmente en tronco y extremidades respetando palmas y plantas. Cerca de la mitad de los lesiones presenta rubor localizado, prurito y ampollas. Su incidencia y prevalencia son desconocidas; sin embargo, su aparición es más común en niños que en adultos. El diagnóstico depende en gran manera de un adecuado examen físico complementado con estudios de laboratorio e histopatología. El tratamiento de elección constituye la administración de antihistamínicos orales. [Ávila JA, Cala JC, Gelvis JA, Torres V, Mosquera H. Urticaria pigmentosa en el adulto. MedUNAB 2012; 15:63-67].

Urticaria pigmentosa is one of the most common forms of mastocytosis, which correspond to a series of processes characterized by an abnormal increase of mast cells in different organs and tissues. Urticaria pigmentosa has an unique manifestation in the skin, characterized by maculopapular lesions 2.5 to 5 mm in diameter, of a color that can range from red and brown, usually distributed on the trunk and limbs respecting the palms and soles. Half of the lesions presented blush on a specific site, pruritus and blisters. The incidence and prevalence are unknown but their appearance is more common in children than in adults. Diagnosis depends on an properly physical examination with laboratory test and histopathological analysis. The treatment of choice is the administration of oral antihistamines. [Ávila JA, Cala JC, Gelvis JA, Torres V, Mosquera H. Urticaria pigmentosa in adult. MedUNAB 2012; 15:63-67].

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.

Placas máculonoduliformes hiperpigmentadas pruriginosas en niño de dos años de edad / Nodular, brownish macules and pruriginous patches in a two years old boy

Se atiende a paciente masculino de dos años de edad, con numerosas placas máculonoduliformes discretamente sobreelevadas, de tinte marronáceo bien definidas, ovaladas e irregulares, muy pruriginosas, localizadas en tronco involucrando especialmente dorso. Tiempo de evolución: desde los seis meses de vida. Se refieren molestas agudizaciones y remisiones parciales, sin desaparición lesional completa a intervalos variables. Se observan episodios ampollosos y de urticación ocasionales, durante la exposición solar y baño corporal con agua caliente. La presentación clínica característica correlacionada con los hallazgos histopatológicos, confirma el diagnóstico de urticaria pigmentosa.

A 2-year old male patient with multiple nodular and brownish itchy macules and patches, located at the back of the trunk was received medical attention at a children´s hospital. Frequent flares and incomplete remissions with blister and weal outbreaks after sun exposure and hot water baths were the rule. The distinctive cutaneous lesions as well as histopathological findings confirm urticaria pigmentosa.

Urticaria pigmentosa: dos presentaciones clínicas diferentes en pediatría / Urticaria pigmentosa: two different clinical presentations in pediatric patients

La urticaria pigmentosa es la forma más común de mastocitosis cutánea. Suele iniciarse de manera bimodal: un pico de incidencia desde el nacimiento hasta los 3 años de vida y otro entre la 2ª y 6ª décadas de la vida. El signo de Darier es constante sobre el área afectada, sin extenderse a piel vecina. La forma de comienzo precoz tiene buen pronóstico y desaparece hacia la adolescencia, pero la de comienzo tardío suele ser persistente o asociarse a afectación sistémica. Se comunican dos casos de urticaria pigmentosa, uno con la descripción clásica de la enfermedad y otro con presentación clínica infrecuente, por lo cual el pediatra debe incorporar ambas formas como manifestaciones distintas de una misma entidad.

Urticaria pigmentosa (UP) is the most frequent clinical feature of cutaneous mastocytosis. It usually begins in a bimodal way: a peak of incidence from birth to the age of 3 and the other one between 2nd and 6th decades of life. Darier's sign is constant over the affected skin without affecting the surrounding skin. When UP starts early, it has a good prognosis disappearing into adolescence, while late onset is often associated with persistent or systemic involvement. This article reports two cases of UP, one with the classic description of the disease and the other with an unusual clinical presentation, prompting the pediatrician to incorporate both forms as different manifestations of the same entity.

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.

Mastocitose / Mastocytosis

A mastocitose é caracterizada pelo acúmulo patológico e ativação de mastócitos nos tecidos e órgãos. Embora a classificação da mastocitose e seus critérios diagnósticos sejam bem aceitos, há necessidade de estabelecer parâmetros para aplicação de testes diagnósticos, avaliação clínica e respostas aos tratamentos. O objetivo deste trabalho é fazer uma revisão extensa da literatura, oferecendo conhecimento global sobre os mecanismos etiopatológicos e isiopatológicos, dando especial ênfase à diagnose, classificação e tratamento da mastocitose, promovendo a educação médica continuada.

Mastocytosis is characterized by pathologic accumulation and activation of mast cells in tissues and organs. Although the classification for mastocytosis and diagnostic criteria are well accepted, there remains a need to define standards for the application of diagnostic tests, clinical evaluations, and responses to treatment. The objective of this article was to make an extensive literature review, providing comprehensive knowledge about the etiopathological and pathophysiological mechanisms, with a special emphasis on diagnosis, classification and treatment of mastocytosis, promoting continued medical education.

A Case of Adult-onset Urticaria Pigmentosa with Bone Involvement / 대한피부과학회지

Urticaria pigmentosa (UP) is the most common variant of cutaneous mastocytosis. Primarily a disease of childhood, in over one-half of the cases onset is before 2 years of age, and in 90%, the disease is confined to the skin. UP precedes the diagnosis of systemic mastocytosis and is more common in adults than in children. Therefore, systemic examination should be performed in patients with UP. We report a case of adult-onset UP with systemic involvement.

A Case of Adult-onset Urticaria Pigmentosa / 대한피부과학회지

Mastocytosis is a heterogenous group of disorders characterized by the proliferation and accumulation of mast cells in a variety of tissues, most often skin. Urticaria pigmentosa is the most common type of cutaneous mastocytosis in both adults and children. However, the clinical manifestation of adult-onset urticaria pigmentosa may have some differences such as smaller macules, more pruritic symptoms, and frequent association with systemic symptoms. We report a case of adult-onset urticaria pigmentosa in a 51-year-old woman which we compare to those cases reported in the literatures.

Urticária pigmentosa em lactentes: relato de um caso e revisão da literatura / Urticaria pigmentosa in infants: a case report and revision of literature

Paciente pediátrico, 6 meses, masculino, internado no Hospital Geral em Caxias do Sul. Apresentou lesões hipercrômicas maculopapulares descamativas, vermelho-acastanhadas, intensamente pruriginosas, atingindo grande parte do corpo. Recebeu anti-histamínicos e banhos de água e aveia, enquanto internado. Foi realizada biópsia de pele, que constatou quadro compatível com urticária pigmentosa (UP). A UP é a mais freqüente manifestação de mastocitose, ocorrendo na pele e caracterizando-se por lesões cutâneas com pigmentação persistente e pruriginosa. A mastocitose é caracterizada por um acúmulo local ou sistêmico de mastócitos, sendo que os sintomas, dentre os quais o principal é o prurido, resultam da liberação maciça de histamina e outros mediadores pelos mastócitos. O diagnóstico é baseado basicamente na história e apresentação clínica, com confirmação de envolvimento cutâneo por biópsia. Ainda não há tratamento definitivo e eficaz, e somente alívio sintomático pode ser atingido (AU)

Pediatric patient, 6 months old, male, admitted to General Hospital in Caxias do Sul. The patient displays hyperchromic maculopapular peeling lesions, reddish-brown, with intense itching extending over the majority of the body. The patient received anti-histamines and oat water baths while at the hospital. The results of a skin biopsy were indicative of urticaria pigmentosa (UP). UP is the most common manifestation of mastocytosis, occurring in the skin and characterized by cutaneous lesions with persistent and itching spots. Mastocytosis is characterized by a local or systemic accumulation of mast cells, accompanied by symptoms, among which the primary one is itching, resulting from the massive release of histamine and other mediators by the mast cells. Diagnosis is based basically on clinical history and examination, confirmed by cutaneous biopsy. There is still no satisfactory and efficient treatment, and only the symptoms can be alleviated (AU)