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Generalized lymphatic anomaly (GLA), previously known as lymphangiomatosis, is a rare developmental disease characterized by abnormal proliferation of lymphatic vascular structures that may involve the dermis, soft tissue, bone, and visceral parenchyma. Being an uncommon condition and the lack of specific symptoms often result in a delayed diagnosis or even misdiagnosis, which, in addition to its progressive nature, can lead to dysfunction of vital organs, and ultimately, a poor prognosis. In this report, we present a unique case of GLA in an upper Egyptian female child. Increasing awareness of the possible phenotypic presentations of such anomalies can lead to early diagnosis and possibly more effective management before significant organ damage ensues. [ABSTRACT FROM AUTHOR]

Meningioangiomatosis (MAM) remains a poorly understood lesion responsible for epileptic disease. In the past, MAM was primarily described in the context of neurofibromatosis type 2 before being mainly reported sporadically. Moreover, the malformative or tumoral nature is still debated. Because a subset of MAM are associated with meningiomas, some authors argue that MAM corresponds to an infiltration pattern of these tumors. For these reasons, MAM has not been added to the World Health Organization (WHO) Classification of Central Nervous System Tumors as a specific entity. In the present study, we characterized a series of pure MAM (n = 7) and MAM associated with meningiomas (n = 4) using histopathology, immunohistochemistry, genetic (fluorescent in situ and DNA sequencing analyses), and epigenetic (DNA‐methylation profiling) data. We evidenced two distinct morphological patterns: MAM with a fibroblastic‐like pattern having few lesional cells, and MAM with a more cellular pattern. A subset was associated with the genetic alterations previously reported in meningiomas (such as a KMT2C mutation and a hemizygous deletion of chromosome 22q including the NF2 gene). The DNA‐methylation profile, using a t‐distributed stochastic neighbor embedding analysis, evidenced that MAM (pure or associated with meningiomas) clustered in a separate group from pediatric meningiomas. The present results seem to suggest that MAM represents a neoplastic lesion and encourage the further study of similar additional series so that it may be included in a future WHO classification. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Proteus syndrome is a rare genetic disease characterized by an asymmetrical growth of individual parts of the body and has only been described in single cases. This patient presented with recurrent manifestations of a laryngeal and hypopharyngeal lymphangioma, which were treated with laser surgery, systemic therapy, and sclerotherapy. The reported data depict the diagnosis and treatment in the department of otorhinolaryngology, head and neck surgery of the university hospital Heidelberg from 2019 until May 2023. The recurrent endoscopy of the upper airway was performed using a flexible HD‐endoscope and the Visera Elite video tower from Olympus, Hamburg. The 29‐year old female patient initially presented in February 2019 with stridor and exertional dyspnea due to a lymphatic malformation of the left larynx and hypopharynx. In April 2019 there was no improvement by sclerotherapy with Picibanil, so that systemic therapy with the PIK3CA inhibitor alpelisib was initiated (03–07/2020) and discontinued due to a high side effect profile. In the course of 2021–2023, three microlaryngoscopies with laser surgical resection and renewed sclerotherapy of the lymphangioma with Picibanil were carried out due to fluctuating findings. After these interventions a stable disease could be established until May 2023. Laser surgical therapy is currently described as the therapy of choice in lymphangiomas in the head and neck region and also showed the highest effectiveness in our patient. In case of airway obstruction in particular, it can bring rapid symptom relief. Alternatively, and with a lower surgical risk, local improvements have been reported by sclerotherapy, which was less effective in the presented case. Rare syndromic diseases require multidisciplinary collaboration. In the case of laryngeal lymphangiomatosis, other treatment options should be considered in addition to surgical excision, especially in the case of recurrence. [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]

Giant congenital melanocytic nevi (GCMN) can be cosmetically significant and can lead to melanoma. There is no standard pharmacologic treatment for GCMN. We present the case of an 8‐year‐old female with kaposiform lymphangiomatosis caused by an NRAS mutation whose nevus spilus‐type GCMN improved on oral selumetinib. [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]

Cutaneous reactive angiomatosis, a group of disorders defined by benign vascular proliferation, is associated with a number of systemic processes, including intravascular occlusion by cryoproteins. We report a case of a 64‐year‐old female patient who presented with a 1‐year history of nontender petechiae of the bilateral arms and lower legs. Dermoscopic evaluation showed increased vascularity with a globular pattern. Over a period of months, her findings progressed to erythematous to violaceous plaques with admixed hypopigmented stellate scarring of the bilateral lower extremities, forearms, and lateral neck. Biopsy showed increased thin‐walled, small dermal blood vessels with focal inter‐anastamosis. Some vessels were occluded by eosinophilic globules suspicious for cryoprotein. Subsequent laboratory studies confirmed a diagnosis of type 1 cryoglobulinemia, prompting a bone marrow biopsy that revealed lymphoplasmacytic lymphoma. Herein, we report the fourth case of angiomatosis secondary to intravascular cryoproteins as the initial presentation of an underlying hematologic malignancy. We also present a review of the literature and emphasize the need for thorough initial workup and close and prolonged clinical monitoring for underlying systemic disease in these patients. [ABSTRACT FROM AUTHOR]

Erythema ab igne is an uncommon physical dermatosis that presents with localized patches of reticulated erythema and hyperpigmentation corresponding with the underlying dermal venous plexus. The rash occurs in response to chronic heat exposure that does not meet the threshold for thermal burn of the skin. The histopathologic findings are characterized by atrophy and thinning of the epidermis, focal hyperkeratosis, and keratinocyte atypia. The dermis displays dilated capillaries, evidence of pigment incontinence, and prominent elastotic material. We report a case of a 65‐year‐old male who presented to his primary care physician with a 1‐year history of reticular erythema and hyperpigmentation with focal ulceration on his right lateral leg. Histopathology on biopsy revealed mild hyperkeratosis and focal epidermal atrophy; however, the most striking finding was a proliferation of dermal vascular spaces lined by pleomorphic endothelial cells and numerous mitotic figures, which was morphologically compatible with angiosarcoma. However, clinicopathologic correlation and immunostaining revealed an actual diagnosis of erythema ab igne with reactive angiomatosis. Reactive angiomatosis—morphologically mimicking angiosarcoma—is a rarely reported feature of severe erythema ab igne, and dermatopathologists should be aware of this possibility to avoid misdiagnosis of erythema ab igne as angiosarcoma. [ABSTRACT FROM AUTHOR]

Bacillary angiomatosis (BA) is an uncommon systemic disease caused by Bartonella henselae (BH) or Bartonella quintana (BQ) that occurs primarily in immunocompromised hosts. Few cases of BA recipients have been reported in adult solid transplant recipients over the years, with most cases presenting years after transplant. We describe a case of a kidney transplant recipient who developed cutaneous BA very early in the post‐transplant period despite not having any exposures. Retrospective testing of donor and recipient's serum was performed and raised the concern for possible donor‐derived infection. A literature review encompassing 1990 to present was also performed in order to better understand the clinical presentation, diagnostics and therapeutic approach of this unusual disease. Combined serology, histopathology and molecular testing (polymerase chain reaction [PCR]) were useful in diagnosing BA in our patient as serology alone might be unreliable. Macrolides or doxycycline for at least 3 months is the recommended therapeutic strategy; however, the optimal duration of treatment is not well established in transplant recipients. In our patient, we decided to use doxycycline for 1 year due to gradual resolution of lesions and ongoing immunosuppression. Patient responded successfully without any documented relapse. [ABSTRACT FROM AUTHOR]

Oral propranolol is recommended as the first‐line agent for infantile hemangioma requiring systemic treatment. Life‐threatening complications such as obstructive infantile hemangioma of the airway or infantile hepatic hemangioma associated with high‐output congestive heart failure are major indications for the consideration of early treatment. We present the case of an infant with life‐threatening diffuse neonatal hemangiomatosis, including airway obstruction due to subglottic hemangioma, heart failure due to multiple hepatic hemangiomas with portohepatic venous shunts, and severe anemia due to continuous gastrointestinal bleeding, in which treatment with intravenous propranolol proved successful. [ABSTRACT FROM AUTHOR]

Past history of COVID-19 infection was present in 4 patients (2-3 months back), while 3 patients had a history of dengue infection before vaccination. Clinical, histopathological, and dermatoscopic characterization of eruptive pseudoangioma developing after COVID-19 vaccination - A case-series Keywords: COVID-19; COVID-19 vaccine; eruptive pseudoangioma; eruptive pseudoangiomatosis; SARS-CoV-2 EN COVID-19 COVID-19 vaccine eruptive pseudoangioma eruptive pseudoangiomatosis SARS-CoV-2 1799 1801 3 05/24/22 20220501 NES 220501 ETHICS APPROVAL Due ethical approval was taken from institutional ethics committee. [Extracted from the article]

We describe a unique case of skeletal and extraskeletal angiomatosis complicated by Kasabach–Merritt syndrome. The patient was a 3‐year‐old boy, who presented with involvement of both femurs and left tibia, as well as with soft tissue lesions of the left thigh. At birth, multiple hemangiomas of the soft tissues of the frontal and parietal scalp had been identified, together with a space‐occupying lesion of the lung. Histologically, the skeletal and soft tissue lesions consisted of a proliferation of thin‐walled, dilated blood vessels, with an endothelial lining devoid of atypia and exhibiting immunoreactivity for CD31 and CD34, while podoplanin and GLUT1 were negative. Whole exome sequencing performed on samples from the lesion of the femur, the tibia and the skin of the thigh, showed a GNAQ (c.286A>T:p.T96S) variant in all specimens, that was confirmed with digital droplet PCR. This case expands the clinical and pathologic spectrum of vascular proliferations showing similar molecular biology, characterized by GNAQ, GNA11 or GNA14 mutations. [ABSTRACT FROM AUTHOR]

Aim: Angiomatosis of soft tissue (AST) is a rare, high‐flow, intramuscular vascular anomaly. In the context of PTEN hamartoma tumour syndrome (PHTS), this AST is referred to as PTEN hamartoma of soft tissue. Given that AST is observed in patients with no history of PHTS, we hypothesised that non‐syndromic AST arises as a consequence of a somatic mutation. Methods and results: Thirteen patients with histologically confirmed AST were retrospectively studied. Details of the patients' personal and family medical histories and symptoms were retrieved from their medical records. The histological analyses were reviewed and a tissue sample was used for genetic testing. Somatic mutations in the PIK3CA gene (p.Glu542Lys; p.Glu545Lys; p.His1047Arg) were identified in the tissue samples from seven patients, all of whom had unremarkable medical histories and had presented with a single lesion located in the lower limb. Five pathogenic variations in the PTEN gene (mutations: p.Lys263Arg; c.1026+2T>A; p.Ala126Thr; p.Leu108Arg; deletion, log ratio −0.55) were identified in the lesions of four patients; two of the latter had multifocal lesions. All four patients displayed macrocephaly, three boys presented with penile freckles, but none had a family history of PHTS. There were no histological differences between the PIK3CA and PTEN groups. Conclusions: AST can be related to either PTEN or PIK3CA mutations and may be multifocal in PHTS. AST appears to be a manifestation of PHTS that occurs in early childhood. The patient's medical history and clinical presentation should prompt the physician to perform specific genetic testing. [ABSTRACT FROM AUTHOR]

Background: Although diffuse dermal angiomatosis (DDA), a rare acquired reactive cutaneous vascular disorder, has been previously reported in association with calciphylaxis (CP), the clinical significance of this relationship has not yet been elucidated. Methods: A total of 24 cases of CP diagnosed from 2013 to 2018 were retrospectively reviewed for the presence of associated DDA. Pertinent clinical information for each patient was also collected, and statistical analysis was performed using multivariable logistic regression, Student t test and Fisher exact test. Results: African American race and comorbid congestive heart failure were the only variables that demonstrated independent, statistically significant association with the presence of DDA. End‐stage renal failure, diabetes mellitus, immunosuppressive and hypercoagulable states, arrhythmia, body mass index, hypertension, coronary artery disease, patient age, duration of CP symptoms, gender, time interval from biopsy to death, anticoagulation therapy and sodium thiosulfate administration at the time of biopsy did not demonstrate a statistically significant association with DDA. Conclusion: DDA does not appear to be associated with disease severity or prognosis in cases of CP; however, in our population CP with concurrent DDA was more prevalent in African Americans and individuals with congestive heart failure. [ABSTRACT FROM AUTHOR]

Keywords: hemangiomatosis; immune thrombocytopenic purpura; pregnancy EN hemangiomatosis immune thrombocytopenic purpura pregnancy 714 715 2 01/19/23 20230201 NES 230201 INTRODUCTION Immune thrombocytopenic purpura is an autoimmune disease characterized by low platelet count.[1] Angiomatosis is generally seen in liver, bone, and soft tissue, so pulmonary angiomatosis is a rare condition in which pleural involvement is extraordinary.[2] CASE PRESENTATION A 37-year-old woman at 31 weeks of pregnancy was admitted because of dyspnea. Chest high-resolution computed tomography reported pleural and pericardial effusion associated with pleural thickening, precordial nodularity in addition to mediastinal and retropectoral lymphadenopathies (Figure 1). In our patient the severe thrombocytopenia responded to pregnancy termination, splenectomy, and corticosteroid therapy. [Extracted from the article]

Summary: Pulmonary complications are frequent in patients with sickle cell disease (SCD), but few studies have described lung pathology in SCD. We studied the lung tissue of 30 deceased SCD patients (1994–2012). Demographics, genotype, clinical characteristics, cause of death and associated conditions are presented. We quantified the presence of pulmonary arterial changes, thrombosis and venous thickening. Alveolar capillary abnormalities were demonstrated using CD34 expression and confocal microscopy. Autopsy and echocardiography reports were reviewed to classify heart abnormalities. Tissue expression of markers of endothelial activation (vascular cell adhesion molecule 1, intercellular adhesion molecule 1 and vascular endothelial growth factor) was quantified in pulmonary vessels. Median age was 33 years; genotype was SS in 19, SC in 7 and Sβ in 4, and there were 18 males. Hypertensive arterial changes were present in 76% of the patients, recent thrombosis in 80% and old thrombosis in 43%. Venous thickening was present in 23% and pulmonary capillary haemangiomatosis foci in 87%. Ten percent of the patients presented right ventricular hypertrophy. There was no increased expression of endothelial activation markers when compared to controls. SCD affects the whole pulmonary vascular tree and reflects the multiple burden on lung vasculature imposed by the disease upon time. [ABSTRACT FROM AUTHOR]

Summary: The recent adoption of an improved lymphatic access technique coupled with Dynamic Contrast‐enhanced Magnetic Resonance Lymphangiography has introduced the ability to diagnose and treat severe lymphatic disorders unresponsive to other therapies. All pediatric patients presenting for lymphatic procedures require general anesthesia presenting challenges in managing highly morbid and comorbid conditions both from logistical as well as medical aspects. General anesthesia is used because of the procedural requirement for immobility to accurately place needles and catheters, treat pain secondary to contrast and glue injections, and to accommodate additional procedures. We reviewed a one‐year cohort of all pediatric patients in a newly created Center for Lymphatic Imaging and Intervention at a tertiary care children's hospital presenting for lymphatic procedures. The patients ranged in age from 4 days to 17 years and weighed from 2.5 to 92 kg. There were 106 anesthetics for 68 patients. Patients were functionally impaired (98% ASA 3 or 4) and included significant comorbidities (79.4%). Concurrent with lymphatic imaging and intervention additional procedures were frequently performed (76%). They included cardiac catheterization, bronchoscopy, endoscopy, and drain placement (thoracic or abdominal). Paralysis and controlled ventilation was used for all interventions. Reversal of paralysis and tracheal extubation occurred in all patients not previously managed by invasive respiratory support. All patients having an intervention were admitted to intensive care for observation where escalation of care or complications (fever, hypotension, bleeding, or stroke) occurred in 25% in the first 24 hours. [ABSTRACT FROM AUTHOR]

Angiomatosis refers to a vascular tumour consisting of several vascular, blood-filled cavities with a lining of well-differentiated endothelial cells. This condition is seen in very young cattle as a malformative process and has been documented in other animals as well. In horses, this disease has only rarely been described, with no reported lesions being found within the equine orbit. This report details the clinical findings, surgical exploration, histopathology, and long-term follow-up of an aged horse with orbital angiomatosis. [ABSTRACT FROM AUTHOR]

Birt-Hogg-Dubé syndrome ( BHDS), first described in 1977, is a rare autosomal dominant disorder, linked to germline mutations in the FLCN (folliculin) gene. Patients may present with different skin tumors, pulmonary cysts with recurrent spontaneous pneumothorax, and renal cancers, but it has also been estimated that about 25% of carriers older than 20 years do not show skin involvement. So far, besides the triad of skin lesions of the original description (fibrofolliculomas, trichodischomas and acrochordons), a wide range of neoplastic and non-neoplastic skin conditions have been reported, i.e. melanomas, trichoblastoma, neural- and connective tissue tumors, lipomas, angiolipomas and focal cutaneous mucinosis. We describe a patient with BHDS developing multiple skin angiomatous lesions with prominent signet-ring features, an association never reported so far. As renal carcinomas represent the most threatening complication in BHDS and the identification of the patients with BHDS is mainly based on the clinical and histopathologic identification of the diagnostic skin lesions, the role of the dermatologist can be crucial in the prevention and early detection of a potentially aggressive renal cancer. [ABSTRACT FROM AUTHOR]

Objective To describe a case of hemoperitonium in a dog with Bartonellosis and peliosis hepatis (PH) lesions that resolved following antimicrobial therapy. Case Summary A 3-year-11-month-old 22.5 kg female spayed mixed breed dog presented for progressive lethargy and vomiting. An abdominal ultrasonographic examination revealed moderate ascites, which when sampled was nonclotting hemorrhagic fluid. An exploratory laparotomy revealed a large volume of nonclotted blood in the dog's abdomen and blood-filled vesicular lesions dispersed diffusely along multiple lobes of the liver. Biopsies revealed lesions indicative of PH. Serology testing for Bartonella species was positive. Treatment with azithromycin resulted in Bartonella serology negative status and no further evidence of hemoperitonium at recheck examination 12 months after initial presentation. New or Unique Information Provided This is the first reported case of PH and hemoperitoneum in a Bartonella species serology positive dog wherein treatment with azithromycin resulted in serology negative status. There have been no subsequent episodes of hemoperitoneum in the 12 months since treatment. [ABSTRACT FROM AUTHOR]

Background Nodular regenerative hyperplasia ( NRH) is a rare histological disorder associated with a wide variety of systemic diseases. Aims We aimed (i) to report the prevalence of NRH in a database of liver biopsies ( LBs) and the frequency of portal hypertension ( PHT) at diagnosis, and (ii) to investigate whether associated diseases and/or specific histological lesions, including abnormalities of the microvasculature, were related to PHT. Methods Patients with a histological diagnosis of NRH, referred by seven clinical departments, were retrospectively selected. Clinical, biological, radiological, haemodynamic and endoscopic data at diagnosis were recorded. LBs were reassessed for microvascular abnormalities. Results NRH was diagnosed in 4.4% of LBs ( n = 159, male: 52%, mean age: 54). Among patients referred for unexplained liver enzyme abnormalities, 15% had NRH. PHT was present at diagnosis in 45 patients (38%), including 13 with portal thrombosis; 65% of patients had an associated disorder. Obliteration of portal vein branches, observed in the LBs of 17 patients (11%), was significantly associated with PHT ( P = 0.02). Periportal angiomatosis, observed in 101 patients (63%), was associated with the absence of PHT ( P < 10−4). Conclusion We suggest that NRH is a frequent histological lesion in the setting of unexplained liver enzyme abnormalities. PHT is present at the time of diagnosis in 1/3 of patients regardless of the presence of associated disease. The frequency of periportal angiomatosis in NRH without obliteration of portal vein branches, and its association with the absence of PHT suggest that obstructive portal venopathy would not represent the most frequent mechanism involved in NRH. [ABSTRACT FROM AUTHOR]

Unlabelled: Haemangiomas represent the most common tumour of infancy. Although most cases are cutaneous benign lesions, multiple skin haemangiomas are associated with visceral involvement, especially of the liver. Hepatic haemangiomatosis may be complicated by high-output cardiac failure due to high-flow arteriovenous connections within the lesions. Different therapeutic strategies for treating haemangiomatosis causing heart failure include medical, surgical and interventional modalities. This study aimed to review the treatment options, discuss their benefits and flaws and propose a practical therapeutic approach for this medical situation.Conclusion: Our approach incorporates heart failure medications, dietary support and propranolol as first-line treatment, while corticosteroids, vincristine, percutaneous intervention and surgery are reserved for refractory cases. [ABSTRACT FROM AUTHOR]

The article discusses the case of an 81 year old man presented with a 5-month history of an asymptomatic, violaceous, poorly demarcated 6 cm plaque with a livedoid appearance over the right deltoid area. The patient was diagnosed with a severe degenerative osteoarthritis of the right shoulder who developed associated secondary intralymphatic histiocytosis, a rare benign cutaneous condition.

The article presents case study of one-and-half-year old boy who was referred for pulsed-dye laser (PDL) treatment for port-wine stain on his left thigh and how the parents reported an episodic increase in erythema accompanied by sweating. It mentions that skin biopsy from the plaque showed papillomatosis and irregular acanthosis of epidermis. It also mentions that diagnosis of eccrine angiomatous hamartoma was based on clinicopathological correlation.

We present the case of a 49-year-old man with an 18-month history of an intermittently pruritic red-brown reticulate eruption of the medial legs, with occasional blistering. There were areas of scale, crust and superficial erosions. The patient worked long overnight shifts standing in front of hot machinery wearing shorts, and spent time sitting in front of a heater at home. A clinical diagnosis of erythema ab igne ( EAI) was made. This settled with heater avoidance and protection of the legs with long trousers, as well as a topical corticosteroid. Histology showed features of cutaneous reactive angiomatosis, a rare condition with various associations but only previously reported once in association with EAI. [ABSTRACT FROM AUTHOR]

Aim The differential diagnosis of hypervascular hepatocellular nodular lesion includes hepatocellular carcinoma and it is sometimes difficult to image. We report herein two patients with hyperplastic hepatocellular nodule associated with localized hemangiomatosis. Methods A hypervascular hepatic nodule approximately 10 mm in diameter was incidentally detected in a 79-year-old woman and a 58-year-old man. Hepatocellular carcinoma was suspected and partial hepatectomy was performed. Results Hepatitis viral markers and tumor markers were negative in both patients. On histology, the nodular lesions had an ill-defined border and included hemangioma-like vessels and sinusoidal dilatation showing immunoreactivity for CD34. There were no abnormal unpaired arteries or a central stellate scar suggesting focal nodular hyperplasia. Hepatic columns in the lesion were thickened to two to three cell layers and cellular density was mildly more increased than the background liver. Cellular atypia and diminished reticulin fibers around the hepatic column were not observed. A survey of the background livers of 13 patients with cavernous hemangioma disclosed similar hemangioma-like vessels in hepatic parenchyma in six patients (46%), but similar nodular lesions were not detected in any patients. Conclusion Taken together, the hepatic lesions in these two patients may be hither-to unrecognized types of hyperplastic hepatocellular lesion associated with localized hemangiomatous lesion that may cause irregular blood flow. [ABSTRACT FROM AUTHOR]

Children with tuberous sclerosis complex, Sturge-Weber syndrome, and Rasmussen encephalitis all have complex but differing needs in the process of transition/transfer to adult care. All three may be associated with long-term normal intelligence or a varying degree of intellectual disability. In tuberous sclerosis complex, the emphasis of care in adulthood shifts from seizure control and developmental issues to renal and psychiatric disease and other issues. In Sturge-Weber syndrome, the emphasis shifts from seizure control and rehabilitation to management of disability and migraine. In Rasmussen encephalitis, transition may be particularly complex for those with adolescent onset. Those successfully operated on for childhood onset have a static problem and the potential to do well in life. [ABSTRACT FROM AUTHOR]

Introduction Diffuse dermal angiomatosis is an entity in the spectrum of reactive angiomatoses characterized by erythematous plaques that mainly affect the lower extremities of patients with a personal history of peripheral vascular disease. Involvement of the breast is a rare event that has only been described as single cases in women with large breasts. Objective Our main aim is to report three rare cases of diffuse dermal angiomatosis of the breast and to better define their clinical, histopathological, and immunohistochemical characteristics. Comorbidities and management will also be discussed. Methods A retrospective search of patients with the diagnosis of diffuse dermal angiomatosis of the breast was made. Databases of three hospitals, Hospital 12 de Octubre ( Madrid, Spain), Hospital La Fe ( Valencia, Spain), and Clinica Dermatologica, University of Genoa ( Italy), were included in the analysis. Results Three middle-aged women who were heavy smokers were found. Physical examination revealed several livedoid plaques on both breasts. Painful ulceration over the violaceous lesions was observed in two cases. Histological features included a diffuse proliferation of spindle-shaped endothelial cells with focal small vessel formation occupying the full thickness of the dermis with scanty extravasated erythrocytes, showing positivity for CD31, CD34, and SMA-α and negativity for D2-40. Management was focused on a strict control of comorbidities, especially on the cessation of smoking habit that led to a conspicuous improvement in all patients. Conclusions We describe all those clinicopathologic features that define diffuse dermal angiomatosis of the breast, which should be considered a distinctive variant into the group of cutaneous angiomatoses. In our experience, a strict control of smoking habit must be the first step in the management of this entity. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 76 year-old woman who was presented to the dermatologist with a vascular and solitary appearing nodule, suggesting a lobular capillary hemangioma, involving the upper lip. She was given oral erythromycin, but was replaced with doxycycline due to difficulty tolerating the macrolide. Her biopsy shows a vascular proliferation with a dense neutrophilic infiltrate. The article discusses Bacillary angiomatosis which may occue in the absence of HIV infection.

The aim of this work was to study the vascular endothelial growth factor A ( VEGF- A) pathway and peritumoral brain edema ( PTBE) through comparison of non-angiomatous and angiomatous meningiomas. Meningiomas are common intracranial tumors, which often have PTBE. VEGF- A is an integral part of PTBE formation and angiogenesis, and the capillary-rich angiomatous meningiomas are known for their PTBE. The VEGF- A receptor VEGFR-2 is responsible for the angiogenic effect of VEGF- A on endothelial cells, which is enhanced by the co-receptor neuropilin-1. Forty non-angiomatous, 22 angiomatous meningiomas, and 10 control tissue samples were collected for the study. Magnetic resonance images were available for 40 non-angiomatous and 10 angiomatous meningiomas. Tissue sections were immunostained for CD34, MIB-1, estrogen- and progesterone receptors. ELISA, chemiluminescence, and RT- qPCR were used for VEGF- A, VEGFR-2, and neuropilin-1 protein and mRNA quantification. Angiomatous meningiomas had larger PTBE (695 vs 218 cm3, p = 0.0045) and longer capillary length (3614 vs 605 mm/mm3, p < 0.0001). VEGF- A mRNA, neuropilin-1 mRNA, and VEGFR-2 protein levels were higher in angiomatous meningiomas independently of the capillary length (p < 0.05). Neuropilin-1 protein levels were lower in angiomatous meningiomas (p < 0.0001). The VEGF- A pathway and tumor capillary length may be essential for PTBE-formation in meningiomas. Further investigations of this pathway could lead to earlier therapy and targeted pharmacological treatment options. [ABSTRACT FROM AUTHOR]

Diffuse dermal angiomatosis ( DDA) represents a benign, acquired, reactive proliferation of vessels. DDA is clinically characterized by painful livedoid plaques with central ulceration, and the histopathologic hallmark is diffuse endothelial cell hyperplasia in the dermis. DDA has been rarely reported in association with calciphylaxis, a condition characterized by calcification of arterial walls with accompanying thrombosis and cutaneous necrosis. We present a case of a 72-year-old man with end-stage renal disease on peritoneal dialysis who presented with painful lesions on his legs, and was found to have DDA in the setting of calciphylaxis. The possible pathogenesis linking DDA and calciphylaxis is discussed. [ABSTRACT FROM AUTHOR]

Background Eruptive pseudoangiomatosis (EP) is a rare, benign, spontaneously regressing exanthema of unknown etiology, characterized by an eruption of distinctive erythematous papules. This study was performed to evaluate the etiologic, clinical, and histopathologic characteristics of EP in Koreans. Methods Patients diagnosed with EP during 2005-2010 were included in a retrospective study. Diagnoses were based on clinical findings and confirmed by histology. Biopsies were re-examined. Patient gender, age at onset, disease duration, lesion location, season of occurrence, concomitant diseases, occupation, previous related events, disease course, responses to treatment, and laboratory findings were analyzed. Results A total of 32 patients were identified. Mean age at onset was 50.06 ± 17.12 years. Two patients developed lesions after outdoor activities. Two patients presented with simultaneous occurrences in family members. One patient reported having taken herbal medicine. One subject experienced systemic contact dermatitis caused by eating a lacquer chicken dish just before the lesions developed. All patients had typical erythematous papules measuring 2-5 mm. Lesions were usually located on exposed sites. No prodromal or systemic symptoms were observed. Routine laboratory tests were within normal ranges, except in one patient with known hyperlipidemia. Most patients were treated with oral antihistamine and topical steroid, or oral prednisolone. Eight patients did not receive any treatment. Mean disease duration was 1.64 ± 1.41 months in the treatment group and 1.20 ± 1.24 months in the no-treatment group ( P > 0.05). Conclusions Any clear relationship between EP and exposure to allergens or insect bites has yet to be elucidated. In patients in whom EP occurs on non-exposed sites, drugs and food should be considered as possible causative agents. Intravascular neutrophil infiltration on histology can be helpful in diagnosing EP. [ABSTRACT FROM AUTHOR]

Bartonella henselae, the etiologic agent of cat-scratch disease, causes a well-defined, self-limited syndrome of fever and regional lymphadenopathy in immunocompetent hosts. In immunocompromised hosts, however, B. henselae can cause severe disseminated disease and pathologic vasoproliferation known as bacillary angiomatosis ( BA) or bacillary peliosis. BA was first recognized in patients infected with human immunodeficiency virus. It has become more frequently recognized in solid organ transplant ( SOT) recipients, but reports of pediatric cases remain rare. Our review of the literature revealed only one previously reported case of BA in a pediatric SOT recipient. We herein present 2 pediatric cases, one of which is the first reported case of BA in a pediatric cardiac transplant recipient, to our knowledge. In addition, we review and summarize the literature pertaining to all cases of B. henselae-mediated disease in SOT recipients. [ABSTRACT FROM AUTHOR]

ABSTRACT Diffuse pulmonary lymphangiomatosis (DPL) is a rare disorder involving the intrathoracic lymphatic system from the mediastinum to the pleura. Although DPL is a lymphatic developmental abnormality and is pathologically benign, it has various clinical presentations and a progressive course. We recently studied a case of DPL in a 35-year-old man presenting with cough and hemoptysis with unilateral chylothorax. A chest computed tomography scan showed diffuse interstitial thickening along the peribronchovascular bundles and intralobular septae with pleural and pericardial effusion. Wedge resection of the left lower lung was performed to make a diagnosis. Microscopically, the lesion was characterized by variably sized and increased lymphatic channels of the interlobular septa and subpleural area. The cells lining the lymphatics were immunohistochemically positive for D2-40 and CD34. This patient is still alive without recurrent symptoms 13 months after beginning a low-fat medium-chain triglyceride diet and chemical pleurodesis. [ABSTRACT FROM AUTHOR]

Bacillary angiomatosis ( BA) is a rare vasculoproliferative disorder due to Bartonella henselae ( BH) or Bartonella quintana. It can involve many organs, including the skin, and has been mainly reported in patients with acquired immunodeficiency syndrome. In organ transplant recipients ( OTR), this disorder remains misdiagnosed and therapeutic guidelines are nonexistent. We report 3 cases of BA with skin involvement in OTR and review similar cases from the literature. BA manifests on the skin with violaceous lesions mimicking Kaposi sarcoma, and is associated with fever, lymphadenopathy, and liver, spleen, or lung nodules. Bartonellosis infections in OTR are due to BH, the agent causing cat-scratch disease ( CSD), but BA comprises histologically a prominent vascular proliferation, which is usually lacking in CSD. Cultures and serologic tests are poorly reliable for the diagnosis, which relies on demonstration of BH within the lesions. A history of cat exposure exists in most cases and pediatric OTR are at higher risk. Prevention consists of regular use of a flea-control product in cats and prompt cleaning of scratches. Our cases highlight several original features of this rare condition, which could potentially improve the management of BA in OTR. [ABSTRACT FROM AUTHOR]

Although very rare, mediastinal nodal angiomatosis is an entity radiologists should be aware of to be a differential diagnosis for a hypervascular mediastinal mass lesion. The CT- and MR-morphological features are non-specific and only histopathology can reveal the definitive diagnosis. [ABSTRACT FROM AUTHOR]

The visceral manifestations of lymphatic disorders (lymphangiomatosis and lymphangiectasia) are particularly severe. Any pathology of the lymphatic vasculature, whether superficial or internal, regional, or systemic, is predominated by the appearance of lymphedema, the characteristic form of tissue edema that occurs when lymphatic dysfunction supervenes. Disease manifestations may include dysregulation of body fluid homeostasis, immune traffic impairment, and disturbances of lipid and protein reabsorption from the gut lumen. The appearance of lymphatic edema invokes complex biological alterations. Many of these changes seem to relate uniquely to chronic lymphatic edema, including a profound stimulus to collagen and adipose deposition. Despite the recent advances in our understanding of these disorders, substantial knowledge gaps remain; these gaps inhibit our ability to accurately identify, categorize, treat, and prevent these diseases. Future diagnostic, therapeutic, and reproductive decisions for affected individuals require an accurate knowledge of the clinical and laboratory presentation, mode of inheritance, treatment response, outcomes, and prognosis. [ABSTRACT FROM AUTHOR]

A 10-year old, female, cocker spaniel was presented with a bleeding lesion on the muzzle and nasal planum observed initially as a small raised nodule at 1 year of age. This became clinically more pronounced at 5 years of age, progressively enlarged thereafter and was diagnosed initially as a vascular tumour. On clinical examination, multiple confluent nodules of variable diameter (0.5–1.5 cm) deformed the outline of the nose. Histological examination revealed a progressive angiomatosis with multifocal infiltrative growth of increased dermal vascularization with different sized and type of anastomosing vessels lined by a monolayer of reactive endothelium, often arranged as papillary projections into the lumina. Partial nosectomy, the first therapeutic approach, was unsuccessful and the lesion relapsed within 6 months. However, subsequent laser photocoagulation therapy provided a good cosmetic outcome, no relapse 1 year later, and appeared to provide an effective alternative to ablation surgery. [ABSTRACT FROM AUTHOR]

Caruso S, Mamone G, Marrone G, Milazzo M, Carollo V, Miraglia R, Maruzzelli L, Pasta A, Minervini MI, Spada M, Riva S, Luca A, Gridelli B. Focal liver diseases in neonatal and pediatric liver transplant candidates: a pictorial essay. Clin Transplant 2009: DOI: 10.1111/j.1399-0012.2009.01139.x © 2009 John Wiley & Sons A/S. The aim of this review is to present the wide spectrum of common and uncommon focal liver diseases affecting neonatal and pediatric liver transplant candidates, analyzed using ultrasonography (US), 16- or 64-multidetector row helical CT (MDCT) and 1.5-T magnetic resonance (MR) fast imaging. Correlation of imaging findings and explanted liver or histology is illustrated in representative cases. Associated uncommon congenital anomalies are shown. [ABSTRACT FROM AUTHOR]

Diffuse neonatal haemangiomatosis (DNH) is an uncommon condition characterized by multiple cutaneous and visceral haemangiomas frequently causing severe complications. Corticosteroids constitute the first therapeutic line; however, when they fail, other alternatives are available, provided possible side effects are closely monitored during and after treatment. We present a case of life-threatening DNH, non-responsive to corticosteroids, successfully treated with Vincristine with minor side effects. We conclude that Vincristine is a valid alternative in corticosteroid-resistant DNH. [ABSTRACT FROM AUTHOR]

Background Eruptive pseudo-angiomatosis (EPA) is a rare, relatively newly described cutaneous disorder characterized by the sudden onset of several bright red, angioma-like papules surrounded by blanched halo. Its aetiology is unknown; however, viral infection or mosquito bites have been speculated as possible causes. Objective This study aims to determine the clinical and histopathological features of EPA, and whether it is associated with Epstein–Barr virus (EBV) infection. Methods We conducted a retrospective chart review of 25 EPA cases from 2006 to 2008. In order to determine latent EBV infection, EBV-encoded small RNA (EBER) in situ hybridization was performed in 18 subjects. To determine EPA's distinguishing histological characteristics, we compared the cases with 22 control cases of perivascular lymphocytic infiltration for haematoxylin and eosin, CD3, CD4, CD8, CD31 and c-kit staining patterns. Results The patient sample's female-to-male ratio was 2.1 : 1, and the patients’ age ranged from 5 to 79 years (average 46 years). The lesions appeared during the months of July to September in all but 3 patients. Skin biopsies demonstrated capillary ectasia with perivascular mononuclear cellular infiltrates in the upper dermis. Most patients were otherwise healthy, and routine laboratory results were all normal except in one patient who had diabetes. The skin lesions faded without any treatment in 1–2 weeks. Results of EBER in situ hybridization were all negative. The only histological distinguishing feature of EPA was the presence of intravascular neutrophils, which was found to be present in 19 of the 20 EPA cases (95%), in contrast to only 3 of the 22 control subjects (14%) ( P < 0.0001). Conclusion The sudden onset of lesions during the summer months among our patients supports the ‘paraviral eruption’ concept of this probably underdiagnosed condition. The significant presence of intravascular neutrophils may be a diagnostic clue of EPA in South Korea. [ABSTRACT FROM AUTHOR]

Schwannoma arising within brain parenchyma is a rare lesion, usually found in children. Reported herein is a case of intracerebral schwannoma in a 5-year-old boy, with a review of the English-language literature on the subject, in which 47 cases were found. Few detailed histological reviews of intracerebral schwannoma exist. The tumor had a distinctive plexiform growth pattern, and small aggregates of Schwann cells spread extensively into the surrounding brain tissue along perivascular spaces adjacent to the tumor nodule. Histological differential diagnoses included perivascular schwannosis and meningioangiomatosis. A few intratumoral axons, seen on immunostaining for neurofilament protein, were trapped at the periphery of the main lesion, but there was no evidence of intralesional axons in the multiple nodules of Schwann cell proliferations that extended into the perivascular spaces, suggesting that the lesions are neoplastic. Because Schwann cells are not a natural component of the central nervous system, the origin of intracerebral schwannomas remains unknown. The histology suggests that Schwann cells of the perivascular nerve plexus are a likely site of origin. [ABSTRACT FROM AUTHOR]

Arteriovenous fistulae (AVF) are defined as congenital or acquired abnormal direct communications between an artery and a vein leading to abnormal blood circulation. This report describes an unusual manifestation of acquired peripheral AVF in a cat for which the diagnosis was confirmed by computed tomographic (CT) imaging and three-dimensional (3D) reconstruction. A 10-year-old female spayed domestic shorthaired cat was presented with a 2-month history of nonhealing, crusting, erosive and ulcerative skin lesions on the dorsal right forepaw. Severe chewing and biting, but not lameness, had been reported. Systemic abnormalities were not noted. Histopathology revealed increased numbers of thin-walled and slightly grouped vascular profiles in the superficial and mid-dermis, which were often markedly dilated and partially obscured by prominent hyaline deposits. There were a few pyknotic nuclear fragments and haemorrhages in vascular walls as well as multifocal luminal thrombosis with or without recanalization. Differential diagnoses included progressive angiomatosis with trauma or AVF with secondary regional venous hypertension. Computed tomographic images were acquired using a 16-slice Siemens Somotom Sensation CT scanner, and 3D images were created using the Voxar 3D software. Image reconstruction revealed tortuous aberrant vasculature on the medial aspect of the radius and around the carpus compared to normal vascularization on the contralateral limb. These changes were suggestive of the diagnosis of acquired peripheral AVF. The differential diagnosis for localized, nonhealing, haemorrhagic, crusted, erosive or ulcerative distal extremity skin lesions in cats should include acquired AVF, and diagnosis may be confirmed with contrast-enhanced CT imaging. [ABSTRACT FROM AUTHOR]

Bartonella henselae is the agent of cat scratch disease and bacillary angiomatosis. Blood donors can be asymptomatic carriers of B. henselae and the risk for transmission by transfusion should be considered. The objective of this study was to demonstrate that B. henselae remains viable in red blood cell (RBC) units at the end of the storage period. Two RBC units were split into two portions. One portion was inoculated with B. henselae and the other was used as a control. All units were stored at 4 °C for 35 days. Aliquots were collected on a weekly basis for culture in a dish with chocolate agar, ideal for the cultivation of this agent. Samples were collected on days 1 and 35 and taken for culture in Bact/AlertR blood culture bottles. Aliquots taken simultaneously were fixed in Karnovsky’s medium for subsequent electron microscopy evaluation. Samples from infected bags successfully isolated B. henselae by chocolate agar culture, although Bact/AlertR blood culture bottles remained negative. Bartonella spp. structures within erythrocytes were confirmed by electron microscopy. The viability of B. henselae was demonstrated after a storage period of RBC units. These data reinforce the possibility of infection by transfusion of blood units collected from asymptomatic blood donors. [ABSTRACT FROM AUTHOR]

Aims: To report 16 cases of sclerosing angiomatoid nodular transformation (SANT) of the splenic red pulp . Methods and results: Patients were selected in two phases. An initial group of seven patients was diagnosed with SANT based on the presence of angiomatoid nodules. Sheets of inflammatory fibrosis were found in three patients, resembling inflammatory pseudotumour (IPT); nine further cases of IPT were reviewed. Angiomatoid nodules were detected, leading to the diagnosis of SANT in all cases. The splenic mass (10–150 mm in diameter) was polycyclic, composed of multiple small nodules of loose connective tissue comprising myofibroblasts and a dense network of capillaries as well as some remnants of sinuses. Collagenous fibrosis surrounded them. Bands or large sheets of fibrosis, infiltrated by various inflammatory cells, particularly polytypic plasmacytes, resembling IPT, were present in 10 cases. Conclusions: SANT of the red pulp is a distinct benign pseudotumorous lesion of the spleen characterized by the presence of angiomatoid nodules. We observed such angiomatoid nodules in all our cases of splenic IPT, which were not follicular dendritic cell or myofibroblastic tumours. We therefore recommend careful examination for angiomatoid nodules in all suspected cases of splenic IPT. [ABSTRACT FROM AUTHOR]

To determine the relative contributions of aging and atherosclerosis to vascular stiffness, we studied aortic stiffness, plaque, and elastin in 8-, 16-, 25-, and 34-week-old male ApoE-KO and C57BL/6J control mice (N = 48). Stiffness increased gradually in both strains up to 25 weeks (p < 0.05), and dramatically between 25 and 34 weeks in ApoE-KO (p < 0.001). Aging ApoE-KO demonstrated increased plaque (p = 0.02), medial thickening (p < 0.001), and severe elastin fragmentation (p < 0.001). We conclude that the contribution of aging to vascular stiffness is relatively minor compared with the influence of atherosclerosis. However, the effect of atherosclerosis on stiffness is significant only with advanced stages of plaque formation. [ABSTRACT FROM AUTHOR]

Vascular proliferations of the breast are uncommon but potentially diagnostically challenging lesions. Clinically apparent processes are more likely to be malignant; however, a range of benign entities which must be differentiated from angiosarcoma also exists. This review discusses first, breast lesions of apparent vascular origin, then benign and histologically bland perilobular, cavernous and capillary haemangiomas. Subsequently, more diagnostically challenging, atypical haemangiomas, papillary endothelial hyperplasia, angiomatosis and angiolymphoid hyperplasia with eosinophilia (epithelioid haemangioma) are considered. In addition, lesions with low-grade malignant potential such as haemangiopericytomas and epithelioid haemangioendotheliomas may rarely present in the breast. However, primary angiosarcomas and radiation-associated vascular lesions are reviewed in depth, as these entities are of greatest clinical and pathological significance. [ABSTRACT FROM AUTHOR]

Purpose: Sturge-Weber syndrome (SWS) is a neurocutaneous disorder with vascular malformations of the skin, brain, and eye. SWS results in ischemic brain injury, seizures, and neurologic deficits. We hypothesized that a decrease in quantitative EEG (qEEG) power, on the affected side, correlates with clinical severity in subjects with SWS. Methods: Fourteen subjects had 16-channel scalp EEG recordings. Data were analyzed using fast Fourier transform and calculation of power asymmetry. Blinded investigators assigned scores for clinical neurological status and qualitative assessment of MRI and EEG asymmetry. Results: The majority of subjects demonstrated lower total power on the affected side, usually involving all four frequency bands (delta, theta, alpha, and beta). qEEG asymmetry correlated strongly with neurologic clinical severity scores and MRI asymmetry scores. qEEG data generally agreed with the MRI evidence of regional brain involvement. In MRI-qEEG comparisons that did not agree, decreased power on qEEG in a brain region not affected on MRI was more likely to occur in subjects with more severe neurologic deficits. Conclusions: qEEG provides an objective measure of EEG asymmetry that correlates with clinical status and brain asymmetry seen on MRI. These findings support the conclusion that qEEG reflects the degree and extent of brain involvement and dysfunction in SWS. qEEG may potentially be a useful tool for early diagnosis and monitoring of disease progression in SWS. qEEG may prove useful, in severely affected individuals with SWS, for determining regions of brain dysfunction. [ABSTRACT FROM AUTHOR]

Purpose: To report the results of non-penetrating deep sclerectomy (NPDS) in the treatment of glaucoma associated with Sturge–Weber syndrome (SWS). Methods: We carried out a retrospective case series analysis of patients who underwent NPDS for glaucoma associated with SWS between 1998 and 2003. The control of glaucoma after NPDS, the results of surgery on intraocular pressure, the need for additional medical treatment and surgical complications were studied. Results: Twelve eyes of nine patients, aged 11 days to 24 years, underwent filtering surgery: nine NPDS procedures were performed and three surgical procedures had to be converted to trabeculectomy because NPDS was not technically achievable. The mean follow-up after surgery was 26.3 months (range 6–48 months). Two trabeculectomies were complicated by choroidal effusion, which resolved in both cases. Good control of glaucoma was obtained during follow-up. Conclusions: Non-penetrating deep sclerectomy is transiently efficient in the treatment of SWS-associated glaucoma. Further studies of NPDS for the treatment of glaucoma associated with SWS are warranted. [ABSTRACT FROM AUTHOR]

Eccrine angiomatous hamartoma is a nevoid proliferation of eccrine glands and small vessels. It usually presents as a solitary, slow growing nodule, manifesting at birth or in childhood, which can be accompanied by hyperhidrosis and pain on palpation. We report an occurrence in an otherwise healthy 3-month-old girl who had multiple, asymptomatic, nodular lesions with a linear arrangement localized to the inguinal fold. We also review the literature on adnexal hamartomas of infancy. [ABSTRACT FROM AUTHOR]