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Copyright of Revista Pediatría de Atención Primaria is the property of LUA Ediciones 3.0 S.L. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Cobb syndrome, or cutaneomeningospinal angiomatosis, is a rare condition that affects young adults, and its etiology has not been completely elucidated. It is characterized by a cutaneous sign, or stigma, associated with spinal or intracranial malformations. The symptoms are quite diverse, but, in most cases, the disease presents motor deficit and pain. The present study reports the case of a 48-year-old female patient, who initially sought dermatological medical care for a single skin lesion in the posterior cervical region. During the excision, it was noticed that the lesion had contiguous behavior to the deep anatomical planes, thus requiring the evaluation of the neurosurgical team. The purpose of this report is to describe this rare disease, covering more details about diagnosis and therapy.

Objective: To study the morphology of the placenta in pregnant women with TB Methods: The morphology of the 26 placentas was evaluated in this study, out of which 15 were from women with various forms and localizations of TB (main group), while 11 were from healthy pregnant women (control group). The placentas were evaluated macroscopically. Central and peripheral zones of the placenta and umbilical cord were sampled and embedded in paraffin. Histological slides were stained with hematoxylin-eosin and examined using an Olympus CX-21 microscope with a Universal Infinity Optical System (UIS2). Results: Women in the main group were significantly more commonly underweight (p

Bartonelloses are diseases caused by Bartonella sp., transmitted to humans by blood sucking arthropod vectors. Clinical presentations include bacillary angiomatosis, cat scratch disease and atypical forms. We performed a review of cases of bartonelloses and hematological malignancies published in HIV-negative patients. Terms used were Bartonella or Bacillary Angiomatosis and Leukemia, Lymphoma, Multiple Myeloma, or Cancer. Fifteen cases met our criteria. Clinical presentations included bacillary angiomatosis, chronic fever, chronic lymphadenopathy, osteomyelitis, neuroretinitis, chronic anemia and hepatosplenic peliosis. Fourteen patients were asymptomatic after antibiotic therapy, and one died before antibiotic treatment. Clinicians should be suspicious of Bartonella sp. infections in immunocompromised patients.

Bovine cutaneous angiomatosis (BCA) is a rare condition characterized by vasoproliferative skin lesions mainly affecting cattle in the northern hemisphere. An eight-month-old Holstein heifer showed two skin easy-bleeding nodules bulging from the epidermis in the forehead and close to the right prescapular region. Skin lesions were within the dermis and had an irregular surface and a soft heterogeneous reddish parenchyma composed of numerous variable-sized arterioles, veins, and capillaries surrounded by abundant collagenous fibrous tissue. Immunohistochemical assays evidenced endothelial cells lining proliferative vascular structure immunolabeled for CD31 and Von Willebrand factor, and vascular smooth muscle cells immunostained for smooth muscle actin. All clinical, pathological, and immunohistochemical features observed in the Holstein heifer were hallmarks of BCA. Considering its potential for hereditary spread, BCA must be included in the differential diagnosis of easily bleeding skin nodules that do not respond to routine topical wound treatments to prevent its spread in Brazilian herds. [ABSTRACT FROM AUTHOR]

Dentro de la clasificación de los síndromes de osteòlisis idiopática, la enfermedad de Gorham-Stout ocupa el cuarto lugar. Es un cuadro clínico caracterizado por la destrucción progresiva de tejido óseo y proliferación vascular, con angiomatosis y linfangiomatosis ósea que produce una osteòlisis progresiva del esqueleto, con pérdida de masa ósea en las áreas afectadas. Este artículo tiene como objetivo presentar un paciente pediátrico con síndrome de Gorham Stout atendido en el Hospital Provincial Pediátrico Universitario José Luis Miranda, de Villa Clara. Es una paciente femenina, de dos años de edad, con cuadro febril de una semana de evolución, aumento de volumen en región dorsal y pérdida de peso. Al examen físico se constataron palidez cutáneo-mucosa y lesión nodular indolora de tres centímetros en la región dorsal. Reapareció la fiebre y se observó marcado aumento de volumen en la región dorso lumbar. Mediante tomografía axial computarizada y resonancia magnética se evidenció osteòlisis de cuerpos vertebrales desde D8 a L2 y aumento de las partes blandas adyacentes. Las manifestaciones clínicas e imagenológicas obligaron a descartar etiologías infecciosas y neoformativas. Se descartaron las primeras y por cumplir con los criterios requeridos se concluyó como enfermedad de Gorham. Esta es una rara y peculiar condición patológica músculo-esquelética en la cual el hueso, virtualmente, se desintegra y es reemplazado por tejido conectivo vascular. Su etiología es especulativa, con una presentación clínica muy variable. Los estudios imagenológicos resultan de gran utilidad. Posee un pronóstico indeterminado a pesar de las opciones terapéuticas empleadas. Por ser un síndrome extremadamente raro se decidió la presentación del caso.

Diffuse dermal angiomatosis (DDA) is a cutaneous reactive angiomatosis. Typically presenting as ulcerated, erythematous, violaceous, or purpuric plaques on the breast or lower extremities, DDA is believed to be a reaction to tissue ischemia. Granuloma inframammary adultorum (GIA) is a type of irritant dermatitis of multifactorial etiology, clinically presenting as papules and nodules. Herein, we report an interesting rash presenting as fungiform papulonodules overlying a large violaceous plaque on the left breast. Biopsy revealed an exuberant epidermal proliferation and a diffuse and deep dermal proliferation, consisting of small slit-like blood vessels in between collagen bundles. In light of these clinical and histopathologic findings in the setting of an indurated plaque on a pendulous breast of a woman with multiple risk factors for local tissue ischemia, a diagnosis of concurrent diffuse angiomatosis of the breast (DDAB) and GIA was rendered. This case highlights the critical importance of clinicopathologic correlation in the diagnosis of multiple diagnostic entities.

Sturge-Weber syndrome is a congenital, neurocutaneous disorder, with an incidence of 1 in 20,000 - 50,000 births, characterized by a port wine-colored facial and scalp patch, leptomeningeal angiomatosis and glaucoma, caused by a change in the GNAQ gene on chromosome 9 (9q21.2). It has no clear genetic pattern so there is no direct evidence of hereditary predisposition. Early diagnosis is necessary as there may be complications such as glaucoma, vascular stenosis, epilepsy, neurological and neurocognitive impairment. This article aims to describe the case of a rare syndrome, its clinical manifestations and treatment. Case report: 8-year-old male patient with a history of seizures since 7 months of age associated with Sturge Weber syndrome, treated with oxcarbazepine and phenobarbital. At the age of 3 years, during his hospitalization, he presented several tonic-clonic seizures, cardiorespiratory arrest and was started with advanced CPR for two minutes. Brain angioresonance was performed, which reported angiomatosis in left sigmoid sinuses and encephalic magnetic resonance imaging, which reported left temporal parietal occipital cortical atrophy associated with linear and nodular millimetric calcifications. Thanks to current research it is known that the syndrome is produced by a mutation in the GNAQ gene, however, its etiology is not yet proven. The development of this clinical case provides important information on the onset and clinical course of WSS manifested by facial angioma, glaucoma and seizures at an early age. [ABSTRACT FROM AUTHOR]

The skin represents an indicator of an animal's health status. Causes of cutaneous diseases in fish most often trace back to biological agents. However, fish skin diseases can also arise from a complex interaction of infectious and non‐infectious causes, making it more difficult to identify a specific aetiology. In the period between April and September of the years 2019–2022, four koi carp (Cyprinus carpio koi) from two European countries presented with multifocal, irregularly round, few mm to 1 cm, variably raised cutaneous reddened areas. The fish displayed good general condition. Cutaneous samples, investigated by microbiological and molecular methods and microscopy, did not indicate a primary pathogenic agent. Gross and histological findings of the cutaneous biopsies were consistent with a multifocal/reactive process centred on dermal vessels. The histological features were reminiscent of angiomatosis, a benign proliferative condition affecting the dermal vessels of mammals, including human patients. The clinical–pathological presentation and the dermatologic condition that affected the koi carp are discussed and compared with the veterinary and human literature. [ABSTRACT FROM AUTHOR]

The Sturge-Webber syndrome is a rare genetic condition of the phacomatosis disorders group. Most of the symptoms are linked to nervous, circulatory and cutaneous system. Neuropsychiatric symptoms may be part of the clinical presentation but they are mostly represented by low to moderate cognitive and affective disruptions. Still, in Sturge-Webber children, familial, environmental and educational influences may impact the child's development. This article will present the case of a 15-years old child, addressed by the authorities to the child forensic psychiatric board, after the family declared they are no longer able to control the violent behavior the child expresses. Giving the Sturge-Webber syndrome diagnostic, a proper analysis of discernment and the connection between the somatic disorder and behavioral disruptions should be investigated. Also, the child has been expelled from various educational institution due to severe aggressive manifestations towards teachers, authority and inside the peer group. Multiple organic, psychiatric and psychologic investigation revealed that the boy presented a violent defiant and oppositional behavior, with sadistic elements, with no apparent emotional drive, lacking real remorse and consideration for the true implications of his actions. Discernment was present although the organic pathology may be considered a substrate for the behavioral disorder. Still, the unemotional trait and severe violence seem to be unrelated to the somatic background. The legal and socio-educational implications are important in this case, as the child's responsibility is being refused by educational institutions and the child's family thus, the future social insertion and development prognosis, remains poor. [ABSTRACT FROM AUTHOR]

Rare presentation of pediatric angiomatosis of the paranasal sinus and skull base presenting mimicking juvenile nasopharyngeal angiofibroma (JNA). This is a 16-year-old male who presented to the emergency room with acutely worsening headaches, decreased visual acuity, subjective diplopia on lateral gaze, and a skull base mass centered in the sphenoid cavity. Endoscopic biopsy at an outside facility was aborted due to profuse bleeding. Upon transfer to a tertiary care center, contrast MR demonstrated a heterogeneously and avidly enhancing vascular mass centered around the sphenoid and skull base originating from the internal maxillary artery with significant bilateral extension into the adjacent paranasal sinuses, sella, and cavernous sinus. History of presentation and imaging was suggestive of JNA. Patient underwent preoperative embolization followed by endoscopic endonasal transphenoidal resection with a skull base trained otolaryngologist and neurosurgeon. Final pathology confirmed angiomatosis. This is only the second reported case of paranasal sinus angiomatosis in the literature. Angiomatosis has a high rate of recurrence and failure of timely diagnosis can lead to requirement of repeated surgical intervention. Re-operations are associated with increased costs, patient dissatisfaction, and poorer surgical/clinical outcomes. Because angiomatosis can mimic JNA, hemangiomas, or other vascular tumors, it is essential to maintain a broad differential diagnosis that includes angiomatosis when evaluating sinonasal tumors.

Background: This study describes the management protocol for capillary-venous malformations in pediatric patients and reports the epidemiology of diagnosed and treated cases at the Unit of Odontostomatology of the Aldo Moro University of Bari from 2014 to 2022. Methods: The authors classified the intraoral and perioral capillary-venous malformations by superficial diameter (<1 cm, 1–3 cm, >3 cm) and ultrasonographical depth extension (≤5 mm, >5 mm). All patients underwent pulsed-mode diode laser transmucosal photocoagulation (8–12 W/cm2); those with malformations that were wide (>3 cm) and deep (>5 mm) received intralesional photocoagulation, too (13 W/cm2). The children received general anesthesia based on their compliance and lesions' extension. The follow-up lasted six months. Results: A total of 22 females and 14 males (age range 4–18 years) presented 63 capillary-venous malformations. Five patients with Sturge–Weber syndrome, seven with hereditary hemorrhagic telangiectasia, and five with angiomatosis showed multiple malformations. The authors found no intraoperative or postoperative complications. Seventeen patients with lesions >1 cm and >5 mm deep required multiple laser sessions to heal. Conclusion: The results of the current study support diode laser photocoagulation as the gold standard for the treatment of intraoral and perioral capillary-venous malformations in pediatric patients. [ABSTRACT FROM AUTHOR]

Sturge–Weber Syndrome (SWS) is a neuro-oculo-cutaneous vascular disorder that includes leptomeningeal hemangioma and port wine stains, usually ipsilaterally, with ocular manifestations and extended lesions over the oral cavity. It is an embryonic developmental disorder affecting both mesodermal and ectodermal germ layers, associated with somatic mutation of gene GNAQ. It has a sporadic occurrence of 1:50,000 and no gender predilection. This case report added to the knowledge and understanding of systematic rehabilitation of a child patient with SWS and its associated risk factors in an emergency scenario. A 3.5-year-old girl child with a known diagnosis of SWS presented abscess over the right lower mandibular deciduous teeth and distinct oral features of SWS, requiring emergency endodontic intervention, followed by full mouth rehabilitation. Thus, an in-depth knowledge of the clinical state that helps in the expert assessment of dental interventions, including emotional co-regulation in those children, is essential.

In this study, a clinical case of 8-year-old male, with a history of seizures from 7 months of age associated with Sturge Weber syndrome, under treatment with oxcarbazepine and phenobarbital is presented. At 3 years of age, during his hospitalization he presented several tonic-clonic seizures, cardiorespiratory arrest, for which he began advanced CPR for two minutes. An angioresonance of the brain was performed, which reported angiomatosis in the left sigmoid sinuses and brain magnetic resonance imaging reported left temporal parietal occipital cortical atrophy associated with linear and nodular millimeter calcifications. Thanks to current research, it is known that the syndrome is caused by a mutation in the GNAQ gene, however, its etiology is not yet proven. The development of this clinical case provides important information on the onset and clinical evolution of WSS manifested with facial angioma, glaucoma and seizures at an early age. [ABSTRACT FROM AUTHOR]

Pseudoangiomatous stromal hyperplasia (PASH) is a benign mesenchymal proliferative lesion of the breast. PASH is postulated to be hormonally induced and predominantly occurs in premenopausal women and postmenopausal women on menopausal hormone therapy. Clinical presentation varies from screen-detected lesions to palpable masses. Imaging findings of PASH are nonspecific. The most common mammographic findings are an oval or round circumscribed non-calcified mass or developing asymmetry. On US, PASH is often seen as an oval hypoechoic mass that may be circumscribed and can have an echogenic rim, or, when manifest as mammographic asymmetry, US may show a corresponding non-mass focal area of echogenic tissue. Limited studies have investigated the MRI appearance, with PASH most often manifesting as non-mass enhancement, or, less often, as an oval or irregular mass with persistent kinetics. Histopathologically, PASH can be mistaken for a fibroadenoma or phyllodes tumor and has features overlapping low-grade angiosarcoma. Assessment of radiologic-pathologic concordance is particularly important as PASH is often an incidental finding, adjacent to the targeted lesion at histopathology. Surgical excision or repeat core-needle biopsy is necessary for discordant suspicious cases. After a benign, concordant diagnosis of PASH, the patient may resume routine screening. [ABSTRACT FROM AUTHOR]

BACKGROUND: Sturge-Weber syndrome is a non-inheritable condition which belongs to the spectrum of neurocutaneous disorders, also known as phacomatoses; it is the most common condition after neurofibromatosis and tuberous sclerosis. This syndrome is characterized by capillary vascular changes in the face (port wine stain) and ocular and cerebral capillary and venous malformations. Given its clinical presentation, the diagnosis is suspected by a complete physical exam and confirmed by imaging studies. CLINICAL CASE: This paper reports two cases of children diagnosed with Sturge-Weber syndrome at the age of three and eight months, respectively, both presenting complex seizures as part of the disease debut and port wine stain at the physical examination. Complementary studies carried out and therapeutic approach are described. CONCLUSIONS: Sturge-Weber syndrome has a specific clinical phenotype (facial hemangioma and seizures). This phenotype could be easily seen in Sturge-Weber syndrome patients and could prevent delay of diagnosis. [ABSTRACT FROM AUTHOR]

Sclerodermatous graft‐versus‐host disease (GvHD) is one of the many clinicopathological variants of chronic GvHD. One of the rarest forms of this variant is GvHD‐associated angiomatosis (GvHD‐AA). We describe the case of a 62‐year‐old male with sclerodermatous GvHD who presented, in consecutive years, two different lesions that showed characteristics of GvHD‐AA. The first lesion fitted perfectly with the previously known features of this rare entity. However, the second lesion was more interesting, as the angiomatoid lesion was surrounded by newly appeared adipocytes, something not previously described. The appearance of this peculiar adipose tissue may be explained as related to an important dermal atrophy, as a concomitant appearance of a lipomatous nevus and GvHD‐AA, or, finally, as mature adipose tissue related to a previous inflammatory process, that is, lipomatous metaplasia. Both lesions were diagnosed as GvHD‐AA, and the second one was considered to be associated with dermal lipomatous metaplasia. We also considered whether hypoxia could be related to both lesions. In the present report, we review previously published cases of GvHD‐AA and discuss the different hypotheses that could explain the appearance of metaplasia associated with the second lesion. [ABSTRACT FROM AUTHOR]

Vascular neoplasms of the breast are uncommon and can be challenging to diagnose, particularly in needle biopsies in which it can be difficult to appreciate architectural features. Here, we survey vascular neoplasms of the breast, including perilobular haemangioma, angiolipoma, capillary haemangioma, venous haemangioma, cavernous haemangioma, sinusoidal haemangioma, angiomatosis, atypical post-radiation vascular proliferation, radiation-associated angiosarcoma, and primary mammary angiosarcoma. We emphasise and illustrate key morphologic features, as well as diagnostic pitfalls and recent updates in our understanding of the molecular genetics of these tumour types. [ABSTRACT FROM AUTHOR]

Diffuse cutaneous angiomatosis is one of the reactive angiomatoses. Historically, it is associated with lesions located in the lower extremities. Breast localization is uncommon, but may be under-reported. We report a non-smoking woman who exhibited this vascular condition of both breasts.

A 1-year-old castrated mixed-breed dog presented with diffuse, purple lesions arranged in an irregular patchy pattern, with a slight elevation on the right hindlimb extending from the tarsus joint to the upper region of the thigh. Dermatological examinations and fungal and bacterial cultures revealed no infectious agents. The therapeutic response to antibiotics and antifungal agents was negative. A histopathology examination of the lesion revealed vascular proliferation with vasodilation and numerous varying-sized vessels. Mast-cell-dominated perivascular cuffing was also noted. The dog was diagnosed with cutaneous angiomatosis due to diffuse lesions and the histopathology findings of hemangioma. [ABSTRACT FROM AUTHOR]

A 10‐month‐old, male entire Rhodesian Ridgeback was presented with a 1‐week history of vomiting, small and large intestinal diarrhoea, hyporexia and weight loss. Physical examination revealed a palpable abdominal mass. Abdominal ultrasonography and contrast computed tomography demonstrated multiple cystic masses of varied size and wall thickness with no appreciable origin. An exploratory laparotomy revealed multiple mass lesions throughout the mesentery, located adjacent to blood vessels and varying in size from approximately 2 mm up to 13 cm in diameter. In histologic sections, the nodular structures resembled thick‐walled arteries. Immunohistochemistry confirmed positive cytoplasmic immunoreactivity for von Willebrand factor, CD31 and smooth muscle actin. This is, to the authors' knowledge, the first case of disseminated peritoneal angiomatosis with arterial differentiation in the dog. The dog was euthanased due to the progressive nature of the mass lesions. [ABSTRACT FROM AUTHOR]

In disease, blood vessel proliferation has many salient roles including in inflammation, when granulation tissue fills superficial defects, or in the recanalization of an occluded blood vessel. Sometimes angiogenesis goes awry—granulation can be exuberant, and plexiform proliferation of vascular components can contribute to pulmonary hypertension. This review focuses on the diverse manifestations of pathologic vascular overgrowth that occur in the brain, spinal cord, and meninges of animals from birth until old age. Entities discussed include systemic reactive angioendotheliomatosis in which glomeruloid vascular proliferations are encountered in various organs including the central nervous system (CNS). The triad of CNS vascular malformations, hamartomas, and benign vascular proliferations are an especially fraught category in which terminology overlap and the microscopic similarity of various disorders makes diagnostic classification incredibly challenging. Pathologists commonly take refuge in "CNS vascular hamartoma" despite the lack of any unique histopathologic features and we recommend that this diagnostic category be abandoned. Malformative lesions that are often confusing and have similar features; the conditions include arteriovenous malformation, cavernous angioma, venous angioma, and capillary telangiectases. Meningioangiomatosis, a benign meningovascular proliferation with dual components, is a unique entity seen most commonly in young dogs. Last, accepted neoplastic conditions range from lower-grade locally acquired growths like hemangioblastoma (a tumor of mysterious interstitial stromal cells encountered in the setting of abundant capillary vasculature proliferation), the rare hemangioendothelioma, and the highly malignant and invariably multifocal metastatic hemangiosarcoma. Additionally, this review draws on the comparative medical literature for further insights into this problematic topic in pathology. [ABSTRACT FROM AUTHOR]

BackgroundBacillary angiomatosis (BA) is a rare manifestation of infection caused by Bartonella species, which leads to vasoproliferative lesions of skin and other organs. Bacillary angiomatosis affects individuals with advanced HIV disease or other immunocompromised individuals. In sub-Saharan Africa, despite the high prevalence of HIV infection and documentation of the causative Bartonella species in humans, mammalian hosts, and arthropod vectors, BA has only rarely been described.MethodsThree adult patients from Uganda and Kenya with deep purple dome-shaped papules or nodules of the skin underwent punch biopsies for histopathologic diagnosis. The biopsies of all 3 patients were sent to a local pathologist as well as to a dermatopathologist at the University of California, San Francisco.ResultsAll 3 patients were clinically suspected to have Kaposi's sarcoma (KS), and local pathologists had interpreted the lesions as KS in 2 of the cases and nonspecific inflammation in the third. Histologic examination by dermatopathologists in the United States revealed nodular dermal proliferations of irregular capillaries lined by spindled to epithelioid endothelial cells. The surrounding stroma contained a mixed inflammatory infiltrate with lymphocytes, eosinophils, and neutrophils. Extracellular deposits of pale amphophilic granular material were noted in the surrounding stroma. A Warthin-Starry stain highlighted clumps of bacilli, confirming the diagnosis of BA.ConclusionsThese 3 cases, to our knowledge, are the first reports of BA in East Africa in the biomedical literature. Each had been originally incorrectly diagnosed as KS. We speculate BA is underdiagnosed and underreported in resource-poor regions, such as sub-Saharan Africa, that have high endemic rates of HIV infection.

Vascular lesions are uncommonly encountered in the breast and they represent a spectrum of benign, atypical and malignant entities. The mammary angiosarcomas can be primary arising de novo, or most frequently secondary to prior radiation therapy for breast carcinoma. There is significant overlap in pathologic features between vascular lesions and their non-vascular mimics, as well as between benign vascular lesions and their malignant counterparts, resulting in diagnostic dilemma, especially in the core biopsy setting. In this article, we review the clinico-radiological findings, pathologic features, role of ancillary testing as well as the management and prognosis of breast vascular lesions, with emphasis on essential pathologic features and potential diagnostic pitfalls.

Introduction: Sturge Weber Syndrome (SWS), known as encephalotrigeminal angiomatosis, is a rare neurocutaneous disorder and is having a prevalence of 1/20-50,000, live births. The syndrome consists of leptomeningeal angiomas and the angiomas of the face, characteristically in the ophthalmic and maxillary divisions of the trigeminal nerve. The neurological presentations include fits, deficits in cognition, glaucoma, and visual field abnormalities. The overgrowth of the soft tissues and extracranial angiomas can also occur in SWS. Case presentation: We are presenting a rare case of Sturge Weber Syndrome in an infant who presented at an early stage of this disorder. The index case presented to us with a large port-wine stain and right-sided focal fits. The CT scan brain showed subtle gyriform tram-track calcification of the left cerebral cortex and the left-sided cerebral atrophy. Ophthalmological examination showed glaucoma of the left eye. The clinical and radiological criteria were used to make the final diagnosis of SWS. Conclusion: The case is being reported in order to increase awareness among medical professionals. [ABSTRACT FROM AUTHOR]

Hemangiomas are frequent benign vascular tumors originating from the proliferation of endothelial cells and several vascular structures variable in size. In rare cases hemangiomas affect the gastrointestinal system (only 0.05% of intestinal tumors) and mesentery, which is considered an even rarer location.In our research, we present a rare case of mesenteric hemangioma in a 3-month-old female infant, presenting acute symptomatic anemia. The mesenteric hemangioma was diagnosed using ultrasonography and contrast enhanced CT, showing an impressive response to propranolol, which was confirmed with US and blood laboratory tests. The patient did not need any surgical treatment.

Abstract Background We report a case of a patient with a rare clinical condition: cystic angiomatosis presenting as pleural effusion and multiple bone lesions mimicking a metastatic malignant neoplasia. With only about 50 such cases published in the literature, it is important to report the clinical presentation and proposed treatment and to share information about the clinical evolution in these patients. Case presentation We report a case of a 45-year-old white man who presented to our hospital with ventilator-dependent pain. Chest tomography detected pleural effusion and multiple osteolytic bone lesions. Oncologic investigation for metastatic malignant neoplasia was started after exclusion of an infectious process. Imaging examinations revealed diffuse osteolytic lesions as well as cystic lesions of the spleen, with discrete glycolytic hypermetabolism visualized by positron emission tomography. After negative results were obtained by investigation of the primary tumor site and a bone biopsy, a final diagnosis of cystic angiomatosis was made. Conclusions In view of the fact that cystic angiomatosis is a heterogeneous disorder of unpredictable prognosis and uncertain treatment, it is necessary to disseminate new cases so that further studies may be undertaken to obtain further physiopathological findings and an effective treatment.

Bartonella is an infrequent yet important pathogen in immunocompromised patients. Bartonella infections can cause serious morbidity and mortality in people living with human immunodeficiency virus (PLWH), particularly those with advanced immunosuppression. The prevalence of clinically evident Bartonella infections among PLWH is relatively low. Here, we reported a bacillary angiomatosis (BA) case in a homeless HIV-positive patient. A 31-year-old man with acquired immunodeficiency syndrome (AIDS) and advanced immunosuppression, who had discontinued antiretroviral therapy (ART) one year ago, referred to the hospital. At the admission, he had nausea, vomiting, anorexia, weight loss, occasional sputum cough, subjective fevers, and multiple skin lesions. Lesions' biopsies were non-diagnostic for routine bacterial, tuberculosis, and fungal infection. However, the diagnosis of Bartonella quintana was confirmed by serum polymerase chain reaction (PCR). After receiving a long course of antibiotic therapy, skin lesions resolved. The patient had a favorable outcome with supportive care and continuation of ART and doxycycline. While easily treated, an infection due to Bartonella may be clinically unrecognized, if skin lesions are absent or overlooked, and microbiologically unrecognized, if appropriate protocols are not followed. Because the fever caused by Bartonella infection is easily treated, it is essential that suspected clinical signs of Bartonella infection in immunocompromised hosts should be reported to the microbiology laboratory. Bartonella quintana infection can result in a broad range of often non-specific clinical manifestations; therefore, patients must be evaluated for suspected bacteremia, and clinical wariness is required for diagnosis. [ABSTRACT FROM AUTHOR]

El síndrome de Sturge-Weber es un trastorno neurocutáneo, congénito, esporádico e infrecuente que afecta aproximadamente a 1 de cada 20 000 a 50 0000 nacidos vivos y que se relaciona con una mutación genética activadora somática en GNAQ. Clínicamente se caracteriza por la presencia de una mácula en vino de Oporto en la piel de territorio trigeminal, angiomatosis leptomeníngea y glaucoma. Puede asociarse a diferentes manifestaciones clínicas, de las cuales las crisis epilépticas representan la manifestación neurológica más frecuente que se asocia a un deterioro cognitivo importante en estos pacientes. En el presente artículo se realiza una revisión descriptiva de la literatura sobre los aspectos etiológicos, fisiopatológicos, de clasificación, clínicos, diagnósticos y del tratamiento del síndrome de Sturge-Weber.

Gorham-Stout disease (GSD) is a rare entity that destroys the bone matrix resulting mainly in osteolysis, pain and pathologic fractures among a broader clinical picture. We report a case of a 60-year-old female with a sudden discovery of pathologic fractures in the pelvis and the absence of the left femoral head. On biopsy, no cellular atypia was found, instead disturbed bone formation with prominent vascularity with scattered foci of necrosis & osteolysis, which lead to the diagnosis of GSD. Possible differential diagnoses were discussed and excluded. The patient was put on Bisphosphonate that led to a relative improvement in the symptoms. This disease needs a more thorough investigation to identify the key cause, what is beyond the scope of this report. [ABSTRACT FROM AUTHOR]

Copyright of Jornal Brasileiro de Patologia e Medicina Laboratorial is the property of Sociedade Brasileira de Patologia Clinica and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Competing Interests: None disclosed.

A fast growing, circumscribed, unilateral swelling of the right mandible of a juvenile horse was observed. Within few weeks, the continuously growing mass reached dimensions ranging from 7 to 10 cm in diameter and resulted in loss of the first deciduous premolar of the affected side. The animal was euthanized due to lesion progression. Histologically the mandibular swelling consisted of numerous variably sized vascular structures, partly filled with erythrocytes and embedded in a loosely arranged fibrous stroma within the medullary cavity of the mandible. Juvenile mandibular angiomatosis was diagnosed. To the authors' knowledge this is the first description of this rare entity in the mandible of a foal.

A 56-year-old female presented with popliteal venous thrombosis, splenomegaly, and sclerotic bone lesions. Bone marrow biopsy showed fibrosis, proliferation of abnormal blood vessels and lymphatics, bone remodelling, and no significant changes in haematopoietic elements. Following a relatively indolent initial clinical course, one year later she rapidly deteriorated and died of respiratory failure associated with widespread disease. Ingrowth of podoplanin+ lymphatics mixed with CD34+/podoplanin– blood vessels into the bone marrow is a new finding and may be a unique feature of this disease.