Transformación nodular angiomatoide esclerosante del bazo: reporte de caso / Sclerosing angiomatoid nodular transformation of the spleen: a case report

La transformación nodular angiomatoide esclerosante es una patología vascular benigna del bazo, desarrollada a partir de la pulpa roja, de etiología desconocida. Se postula que puede estar relacionada con la enfermedad por inmunoglobulina 4 y la infección por el virus de Epstein-Barr. La mayoría de los casos son asintomáticos, constituyendo hallazgos incidentales en estudios por imágenes. Presentamos el caso de un paciente masculino de 41 años con antecedentes de tiroidectomía por carcinoma papilar que consulta por fiebre. Recibió tratamiento sintomático y se realizó tomografía computarizada de abdomen por síntomas abdominales inespecíficos. La tomografía evidenció una imagen de aspecto sólido, con tenue realce periférico con el contraste que mide 62 por 52 por 51 milímetros en el polo inferior del bazo. Se realizó esplenectomía que midió 14 por 11 por 4 centímetros y pesó 284 gramos. Se identificó una formación nodular sólida, bien delimitada, con área central de aspecto fibroso, con tractos blanquecinos que delimitan áreas violáceas. La microscopía presentó nódulos coalescentes redondeados de aspecto angiomatoide, con proliferación vascular revestida por células endoteliales sin atipia, entremezclados con células ahusadas, infiltrado de linfocitos y macrófagos. El estroma entre los nódulos mostró proliferación miofibroblástica con linfocitos, plasmocitos y siderófagos. Inmunohistoquímica tuvo marcación positiva en los vasos para CD34 y CD31, sectores positivos para CD8 y negativos para CD34. Una célula positiva para inmunoglobulina 4 (IgG4) por campo de gran aumento. El estudio para Epstein-Barr por reacción en cadena de la polimerasa fue negativo. Para el diagnóstico los estudios de imagen son inespecíficos, por lo que la confirmación diagnóstica la da el estudio histopatológico. La esplenectomía es curativa sin casos reportados hasta la actualidad de transformación maligna o recidiva. No se conocen factores de riesgo y no se han comprobado factores desencadenantes, excepto la asociación de casos con IgG4 y virus de Ebstein-Barr. Por ser una entidad patológica recientemente descrita es necesario recopilar series grandes y revisar nuestros archivos, reevaluando algunos de sus diagnósticos diferenciales para lograr una mejor comprensión de la misma.

Sclerosing angiomatoid nodular transformation is a benign vascular pathology of the spleen, developed from the red pulp, of unknown etiology; it is postulated that it may be related to IgG4 disease and Epstein-Barr virus infection. Most cases are asymptomatic, constituting incidental findings in imaging studies. We present a 41-year-old male patient with a history of thyroidectomy for papillary carcinoma who consulted for fever, received symptomatic treatment and performed a computed tomography of the abdomen for nonspecific abdominal symptoms, the same evidence in the lower pole of the spleen a solid-looking image with faint Peripheral enhancement with contrast, measures 62x 52x51 mm. A splenectomy measuring 14x 11x4 cm and weighing 284 grams was performed, identifying a solid, well-defined nodular formation, with a central fibrous-looking area, with whitish tracts that delimited purplish areas. Microscopy: rounded angiomatoid-like coalescing nodules, with vascular proliferation lined by endothelial cells without atypia, interspersed with spindle cells, infiltrated by lymphocytes and macrophages. The stroma between the nodules shows myofibroblastic proliferation with lymphocytes, plasma cells, and siderophages. Immunohistochemistry: positive labeling in vessels for CD34 and CD31, positive sectors for CD8 and negative for CD34. One IgG4 positive cell per high power field. The study for Epstein-Barr by Polymesara Chain Reaction was negative. For the diagnosis, the imaging studies are nonspecific, so the diagnostic confirmation is given by the histopathological study. Splenectomy is curative with no reported cases of malignant transformation or recurrence to date. There are no known risk factors and no triggering factors have been proven, except the association of cases with IgG4 and Ebstein-Barr virus. As it is a recently described pathological entity, it is necessary to collect large series and review our files, reevaluating some of its differential diagnoses to achieve a better understanding of it

Scanning electron microscopy of dermatofibroma

Abstract: Dermatofibroma is a proliferation of spindle cells located in the dermis. We used scanning electron microscopy to examine two histologically confirmed lesions and observed preserved collagen bundles in the perilesional area. In the lesional area, the collagen was denser, without formation of bundles. Higher magnification showed collagen with mesh-like appearance similar to stretched tufts of cotton. Very high magnification evidenced the tufts of cotton and spindle cells measuring 2 to 12 microns.

Case for diagnosis. Atypical fibroxanthoma

Abstract Atypical fibroxanthoma is a rare superficial fibrohistiocytic tumor. Clinically, it presents itself as a painless, solitary, ulcerated, and slow-growing nodule found mainly on sun-exposed areas - especially of the head and neck - of elderly individuals. The diagnosis is based on histopathological and immunohistochemical features. Immunohistochemical staining plays a critical role for the exclusion of differential diagnoses. Since atypical fibroxanthoma has an indolent clinical course, surgical excision is adequate for treatment. We present a case of atypical fibroxanthoma on the ear of a patient.

Dermatomiofibroma lineal: reporte de un caso / Linear dermatomyofibroma: report of a case

El dermatomiofibroma es un tumor benigno infre-cuente de origen mesenquimático. Generalmente, se presenta como un tumor rosado nodular, espe-cialmente en mujeres en la década de los 30, en hombros y axila. Clínica e histológicamente puede simular otros tu-mores mesenquimáticos como el dermatofibroma , leiomioma y el dermatofibrosarcoma protuberans. Se presenta caso de dermatomiofibroma lineal, para describir sus características clínicas e histo-lógicas, necesarias para su correcto diagnóstico y enfrentamiento terapéutico.

Dermatomyofibroma is an uncommon mesen-chymal benign tumor. It usually presents in wo-men at their thirties, as a pink nodular tumor on the shoulder and axilla. Clinically and histologically it may mimic other mesenchymal tumors such as dermatofibroma, leiomyoma and dermatofibrosarcoma protube-rans. We present the case of a linear dermatomyofi-broma and describe its clinical and histological characteristics, which may help the clinician in the correct diagnosis and therapeutic approach of this rare but benign tumor.

Case for diagnosis. Multinucleated cell angiohistiocytoma

Abstract: Multinucleate cell angiohistiocytoma is a rare idiopathic benign fibrohistiocytic and vascular proliferation usually presenting as multiple asymptomatic papules, red to violaceous in colour, primarily located on the extremities of middle-aged females. This entity is probably underdiagnosed due to the lack of recognition by clinicians and pathologists. We describe a patient with a multinucleate cell angiohistiocytoma of the face, a less frequent localization, in order to increase awareness of this entity and elucidate its clinical, histopathological, and immunohistochemistry features.

Patrón dermatoscópico en arcoiris (irisado) en dermatofibroma hemosiderótico: reporte de un caso / Dermoscopic pattern in rainbow (iridescent) in hemosiderotic dermatofibroma: a case report

El patrón en arcoiris ha sido un tema controvertido, dado que en un principio se planteó como un patrón dermatoscópico específico asociado al diagnóstico de Sarcoma de Kaposi. Sin embargo, esta asociación ha sido cuestionada por diversos reportes que han evidenciado la presencia de este patrón dermatoscópico en otro tipo de lesiones, tales como, otros tumores vaculares, cicatriz hipertrófica, angioqueratoma, dermatitis por estasis, pseudo-Kaposi (acroangiodermatitis), melanoma, liquen plano, dermatofibroma hemosiderótico, entre otros. Se ha propuesto que este efecto se genera por la absorción, difracción y difusión de la luz polarizada y su interferencia con diferentes componentes vasculares y fibrosos de la dermis más que por algún hallazgo histopatológico específico. Presentamos el primer caso con registro fotográfico de dermatofibroma hemosiderótico con presencia de un 'patrón irisado' en la dermatoscopía.

The dermoscopic 'rainbow pattern' has been a controversial issue; it was initially proposed as a specific dermatoscopic pattern associated with Kaposi's Sarcoma. However, this has been questioned by several reports that have shown the presence of this dermoscopic pattern in a wide variety of lesions such as other vascular tumors, hypertrophic scars, angiokeratoma, stasis dermatitis, pseudo-Kaposi acroandgiodermatitis), melanoma, lichen planus, and hemosiderotic dermatofibroma, among others. It has been proposed that this effect is generated by the absorption, diffraction and diffusion of polarized light and its interference with different vascular and fibrous components of the dermis, rather than being caused by a specific histopathologic finding. We present the first photographically recorded case of hemosiderotic dermatofibroma, characterized by the presence of a rainbow pattern in dermoscopy.

Dermatomyofibroma

Abstract: We report a case of dermatomyofibroma that, to our knowledge, is the second case reported in Brazil. About 100 cases have been reported worldwide. Dermatomyofibroma represents a rare, benign mesenchymal neoplasm of fibroblastic/myofibroblastic differentiation, with prolonged evolution and little or no symptoms. It most commonly occurs in young women and male children. Dermatomyofibroma can be easily confused with other clinical entities, which could lead to unnecessary treatments. Therefore, it is important that dermatologists and pediatricians suspect and start to consider this hypothesis in their diagnostic exercises.

Dermatofibroma simulating seborrheic keratosis dermoscopically

Abstract: Dermatofibroma is a frequent benign tumor of easy clinical diagnosis in most cases, but that can mimic other dermatoses. Dermoscopy may help to define the diagnosis and its classical pattern is a central white area, similar to a scar, surrounded by a discrete pigment network. However, dermoscopic findings are not always typical. We describe here a case of dermatofibroma exhibiting ridges, furrows and pseudocomedos, a pattern which is typical of seborrheic keratosis, in dermoscopy.

Clinical and histological patterns of dermatofibroma without gross skin surface change: A comparative study with conventional dermatofibroma.

Backg round: Dermatofibroma sometimes clinically presents as a nodular lesion without gross skin surface change. Clinicopathologic features of this variant of dermatofi broma have not been evaluated. Aims: To assess clinicopathologic features of dermatofi broma presenting as a subcutaneous nodule. Methods: This study reviewed the clinical and histological features of 42 cases of subcutaneous dermatofibromas and compared them with 95 cases of conventional dermatofi broma. Results: Dermatofi broma without gross skin surface change was associated with a shorter pre-diagnosis duration than conventional dermatofi broma. Increase in size during the pre-diagnosis period was signifi cantly more frequent in the conventional type. In addition, these dermatofi bromas were more likely than the conventional type to occur in the head and neck region. Although tumor depth was deeper than in the conventional type, less than half of the dermatofi bromas without gross skin surface change were found histologically to be “subcutaneous” or “deep-penetrating dermatofi broma”. Subcutaneous extension was more frequent in these dermatofi bromas while focal stromal hyalinization and hemosiderin deposits were more common in the conventional type. Limitations: This study is a retrospective, single center design. Conclusion: The present study suggests that dermatofi broma without gross skin surface change is a variant type with distinct clinical and histological features that distinguish them from conventional dermatofi broma.

Case for diagnosis

We report the case of an 11-year-old male patient with a histopathological and immunohistochemical diagnosis of dermatofibroma with an atypical clinical presentation on the right forearm. Although dermatofibroma is considered a benign skin tumor, some of its differential diagnoses, such as dermatofibrosarcoma protuberans and malignant fibrous histiocytoma, are truly aggressive. Lesions with atypical clinical aspects and topology associated with specific histopathological variants are some of the criteria for complete tumor excision.

Variants of dermatofibroma - a histopathological study

Several variants of dermatofibroma have been described. They are essentially distinguished by their clinical and histopathological features. To review the mainfeaturesof these variants, a retrospective study of skin biopsies and tissue excisions of dermatofibromasperformed in the dermatology and venereology service at the Hospital Garcia de Orta between May 2007 and April 2012 was carried out. During that period, 192 dermatofibromas were diagnosed in 181 patients, the lesions being more common in women. Median age of the study population was 48 years. The most common lesion site was the limbs (74% of patients). The histopathological types found were common fibrous histiocytoma (80%) and the aneurysmal (5.7%),hemosiderotic (5.7%), epithelioid (2.6%), cellular (2.1%), lipidized (2.1%), atrophic (1.0) and clear cell (0.5%) variants. Based on these findings, this review focuses on the clinical and histological features of the various variants of dermatofibroma in terms of their clinical presentation, distinct histopathological features, differential diagnosis and prognosis.

Bazex Syndrome / Sindrome de Bazex

Acrokeratosis Paraneoplastica or Bazex syndrome is a dermatologic manifestation usually associated with the diagnosis of squamous cell carcinoma of the upper aerodigestive tract. We report a case with exuberant clinical manifestations, exemplifying the typical cutaneous lesions in this rare syndrome, in a patient with squamous cell carcinoma of the esophagus.

Acroceratose paraneoplásica ou síndrome de Bazex é uma manifestação cutânea paraneoplásica rara, geralmente associada a carcinoma de células escamosas do trato aerodigestivo superior. Relata-se um caso com manifestações clínicas exuberantes, ilustrativo quanto às lesões cutâneas típicas da síndrome, em paciente portadora de carcinoma de células escamosas do esôfago.

Congenital multiple clustered dermatofibroma and multiple eruptive dermatofibromas - unusual presentations of a common entity / Dermatofibroma multiplo agrupado congenito e dermatofibromas multiplos eruptivos - apresentacoes singulares de uma entidade comum

Dermatofibroma is one of the most common entities seen in dermatology clinical practice. Several clinical subtypes have nevertheless been described, all of them of uncommon occurrence. The authors present two rare clinical variants of dermatofibromas: congenital multiple clustered dermatofibroma (the presented case is the 4th congenital case to be reported so far) and multiple eruptive dermatofibromas developing in the setting of a Sjögren's syndrome. Since the uncommon subtypes may not be clinically evident, dermatologists should familiarize themselves with their main features and we advise a high level of clinical suspicion in order to reach the correct diagnosis.

O dermatofibroma é uma das entidades mais frequentemente observadas na prática clínica dermatológica. No entanto, além do dermatofibroma comum, vários subtipos clínicos de ocorrência incomum têm sido descritos na literatura. Os autores descrevem duas variantes clínicas raras de dermatofibromas: dermatofibroma múltiplo agrupado congênito (o caso apresentado é o quarto caso congênito reportado até hoje) e dermatofibromas eruptivos múltiplos no contexto de uma Síndrome de Sjögren. Estes diagnósticos menos comuns podem não ser clinicamente evidentes portanto os dermatologistas devem estar familiarizados com estas apresentações, sendo de suma importância um elevado índice de suspeita clínica.

Atrophic dermatofibroma / Dermatofibroma atrofico

Dermatofibroma is a benign fibrohistiocytic tumor, common and easily diagnosed when classical clinicopathologic features are present. The atrophic variant of dermatofibroma is of uncertain origin. This lesion is characterized clinically by a flat or atrophic and depressible surface. Histopathological features show reduction of the thickness of the dermis and elastic fibers. We report a typical case of this uncommon and probably underdiagnosed variant.

O dermatofibroma é um tumor fibrohistiocitário benigno, comum e facilmente diagnosticado quando apresenta os achados clinicopatológicos clássicos. O dermatofibroma atrófico é uma variante específica do dermatofibroma, de origem ainda incerta. Esta é caracterizada clinicamente por lesão plana ou atrófica, depressível à compressão. Ao exame histopatológico, observa-se redução da espessura da derme e redução das fibras elásticas. Relatamos um caso típico desta variante incomum e provavelmente subdiagnosticada.

Dermatofibroma in a black tattoo: report of a case / Dermatofibroma sob pigmento preto de tatuagem: relato de um caso

Tattooing has been associated with a variety of complications including inflammatory and granulomatous reactions, transmission of infections, and neoplasms. We report a case of a 24-year-old male who presented with a 2-month history of an erythematous nodule involving a newly made tattoo on the right leg. An excisional biopsy was performed and the histopathological evaluation was consistent with dermatofibroma. Only three cases of dermatofibroma associated with tatooing were reported in litetature. We report an additional case and review the literature regarding cutaneous reactions to tattoos.

Tatuagens têm sido associadas com uma variedade de complicações incluindo reações inflamatórias e granulomatosas, transmissão de infecções e neoplasias. Relatamos um caso de homem com 24 anos de idade que apresentava há dois meses nódulo eritematoso sob pigmento preto de uma tatuagem na coxa direita. A biópsia excisional foi realizada e a avaliação histológica foi consistente com dermatofibroma. Apenas três casos da associação dermatofibroma e tatuagem foram relatados na literatura. Nós reportamos um caso adicional e revisamos a literatura sobre reações cutâneas em tatuagens.

Estudio clínico-histopatológico e histoquímico del Histiocitoma Fibroso Cutáneo Benigno / Clinical histopathological and histochemical study of Benign Cutaneous Fibrous Histiocytoma

El histiocitoma fibroso cutáneo benignoes un tumor mesenquimatoso frecuente que puede mostrar áreas de diferenciación miofibroblástica. Se lo clasificaba como reactivo o reparativo, pero anomalíascromosómicas en algunos casos, apoyan su naturalezaneoplásica benigna.Objetivo. Analizar la presentación clínico-dermatológica, histopatológica e histoquímica de los histiocitomas fibrosos cutáneos benignos.Diseño. Descriptivo, retrospectivo.Métodos. Se incluyeron 50 casos (N=50) de biopsiasescisionales de piel, con diagnóstico de histiocitoma fibroso, en pacientes de ambos sexos, mayores de 15años, entre el 209 y 2013. Se aplicaron marcacioneshistoquímicas especiales y se valoraron en cruces.Resultados. Los histiocitomas fibrosos se presentarongeneralmente en mujeres (64%), de entre 18 a 87 años.Las lesiones fueron únicas (90%) y se localizaron enextremidades (64%). La variante fibrocolágena fue la másobservada (56%). Las medidas reales del tumor fueronmenores a 0,5 cm (52%). La intensidad de lasmarcaciones citoquímicas para Ácido Periódico de Schif: 24+, Azul de Toluidina: 15 +, Tricrómico de Mason: 19 +.Conclusiones. En este estudio el histiocitoma fibrosocutáneo benigno se presentó como nódulofibrocolágeno, solitario, asintomático, menor a 1 cm.,con predominio de localización en extremidades y mayor frecuencia en el sexo femenino. Destacamos la importancia de los aportes en morfología y componentesintratumorales que otorga el empleo de técnicashistoquímicas para definir el correcto diagnóstico clínico dermatológico.

Background. Cutaneous benign fibrous histiocytoma is afrequent mesenchymal tumor, can also show areas ofmyofibroblastic diferentiation. It was clasifed as reactiveor eparative lesions, but chromosomal abnormalites insome cases, suports its benign neoplastic nature.Objetive. Analyze dermatological, histopathological andhistochemical presentation of cutaneous benign fibroushistiocytomas.Design. Descriptive and retrospective study.Methods. There were 50 cases (N = 50) of excisionaskin biopsy, with fibrous histiocytoma diagnosi, in patientsof both sexes, aged 15 years, betwen 209 and 2013.Special histochemical stains were aplied and evaluatedin croses.Results. The fibrous histiocytoma generaly presents inwomen (64%), beteen 18 to 87 years. The lesions wereunique (90%) and were located in the limbs (64%).Fibrocolagen variant was the most frequent (56%). Realmeasurements of the tumor were les than 0.5 cm (52%).The intensity of the cytochemical stains for Periodic AcidSchif were: 24 +, for Toluidine Blue: 15 +, for Mason'strichrome stain were: 19 + +.Conclusion. In this study cutaneous benign fibroushistiocytoma was presented as a fibrocolagenous solitaryasymptomatic nodule, les than 1 cm., with limbs locationpredominance and more frequently in females. Weemphasize the contributions in morphology andintratumoral components that he use of histochemicaltechniques provides to define the proper dermatologicaldiagnosis.

Fibrohistiocitoma conjuntival simulando pterígio atípico na região amazônica: relato de caso / Conjunctival fibrous histiocytoma simulating atipical pterygium in the Amazon region: case report

Relatamos um caso inédito na literatura brasileira de fibrohistiocitoma conjuntival descoberto durante projeto comunitário de larga escala realizado pela equipe do Centro de Referência em Oftalmologia do Hospital das Clínicas da Universidade Federal de Goiás na região amazônica. Paciente do sexo feminino, leucoderma, de 38 anos de idade, apresentava lesão vascularizada no olho direito no canto medial da conjuntiva bulbar e carúncula com extensão para o limbo e invadindo cerca de 2 mm da córnea medial com configuração semelhante a um pterígio. O exame histopatológico revelou lesão constituída por células fusiformes, com arranjo estoriforme e células histiocitárias gigantes. A imunohistoquímica revelou forte positividade para vimentina e as células neoplásicas foram negativas para AE1/ AE3, HMB 45, proteína S 100. O diagnóstico histopatológico foi de fibrohistiocitoma. A evolução poderá demonstrar o crescimento tumoral e a possibilidade de lesão maligna.

An inedited case in the Brazilian literature of conjunctival fibrous histiocytoma discovered during a wide scale community project accomplished by the team of the Center of Reference in Ophthalmology of the Hospital das Clínicas da Universidade Federal de Goiás in the Amazon region is reported. A 38 year-old caucasian woman presented with a vascularized lesion on the medial portion of the bulbar conjunctiva and caruncular region of the right eye with extension for the limbus and invading about 2 mm of the medial cornea resembling a pterygium. Pathologic findings revealed that the lesion was constituted by spindle-shaped cells, with storiform arrangement, and large histocytelike cells. The immunohistochemistry revealed strong positivity for vimentin and the neoplastic cells were negative for AE1/AE3, HMB 45, S 100 protein. The histopathologic diagnosis was fibrous histiocytoma. The evolution demonstrated the tumoral growth and the possibility of a malignant lesion.