Diffuse dermal angiomatosis dda is a rare, acquired cutaneous, reactive, vascular disorder that was first described in 1994. Case archives sturge weber syndrome encephalotrigeminal. Bacillary angiomatosis is an infectious disease causing proliferation of small blood vessels in the skin and visceral organs of patients with human immunodeficiency virus infection and other immunocompromised hosts. Bacillary angiomatosis is classified as a category b. Bacillary angiomatosis is a systemic disease diagnosed in immunocompromised patients. Dda is clinically characterized by painful purpuric plaque with central ulceration. Cases have been reported in organ transplant recipients, in patients with leukemia and those on systemic steroid therapy. It consists of many angiomas these tend to be cavernous hemangiomas, which are sharply defined, spongelike tumors composed of large, dilated, cavernous vascular spaces. The objective of this article was to offer a better characterization of the typical clinical presentation, radiologic findings, histology, treatment approaches, and differential diagnosis of cystic angiomatosis, a rare condition of which previous reports have been confusing because of unclear diagnostic criteria, different classifications, and variations in terminology. Angiomatosis definition of angiomatosis by medical. Soft tissue angiomatosis develops almost exclusively in the first two decades of life with female predilection and is very uncommon after the age of 30 years. Bacillary angiomatosis in an immunecompetent patient.
The organisms causing bacillary angiomatosis resemble the agent of verruga peruana and oroya fever, bartonella bacilliformis, in producing a histologically similar vascular proliferation, in having a gramnegative wall structure as observed using electron microscopy, and in tending to grow in clumps visible by light microscopy. Sturge weber encefalotrigeminal angiomatosis5 is a neurocutaneous disorder characterized by a clinical triad of leptomeningeal angioma. Glaucoma and neurologic complications, including seizures and. It is one of the phakomatoses and is often associated with portwine stains of the face, glaucoma, seizures, intellectual disability, and ipsilateral leptomeningeal angioma cerebral malformations and tumors. A cause of unbearably painful breast, diffuse dermal. Histologically, diffuse and mostly extravascular interstitial proliferation of cd31positive. Encephalotrigeminal angiomatosis or sturgeweber syndrome. The study of congenital leptomeningeal angiomatosis has been mentioned in research publications which can be found using our bioinformatics tool below. Diffuse dermal angiomatosis genetic and rare diseases. Cutaneovisceral angiomatosis is a rare vascular disorder characterized by generalized multiple, red brown to blue, discrete papules, macules, plaques and nodules ranging in size from millimetres to fig. It is caused by bacterial infection with bartonella quintana and bartonella henselae cause of catscratch disease and also known as rochalimaea henselae.
Angiomatous definition of angiomatous by the free dictionary. Diffuse dermal angiomatosis dda is a rare skin condition that commonly presents as erythematous, violaceous, indurated plaques on the lower extremities of patients with severe peripheral vascular disease. In september 2015, a 36yearold, emaciated, hungarian man presented with a 3month history of widespread, red, rustcolored papules and nodules partially erosive and crusted as well as a 4 cm wide, prominent, skincolored, subcutaneous tumor at the left zygomatic arch fig. This case report is a good illustration of the diffuse dermal angiomatosis variant of reactive angiomatosis. Breast localization is uncommon, but may be underreported. Pathology of bacillary angiomatosis dr sampurna roy md. Additional and relevant useful information for angiomatosis.
Diffuse dermal angiomatosis diffuse dermal angiomatosis draper, b boyd, a 20050101 00. She had a history of seizures and had a port wine stain on the right side of her face, which followed the distribution of the trigeminal nerve. A spectrum of unusual neuroimaging findings in patients. Historically, it is associated with lesions located in the lower extremities.
Retinal angiomatosis american academy of ophthalmology. Angiomatosis encephalotrigeminal symptoms, causes, diagnosis, and treatment information for angiomatosis encephalotrigeminal sturgeweber syndrome with alternative diagnoses, fulltext book chapters, misdiagnosis, research treatments, prevention, and prognosis. There are disseminated multifocal hemangiomas in the skeleton. Skeletal angiomatosis typically presents in two distinct clinical settings. Angiomatosis leptomeningea sin nevus facial y disminucion del. This case is an illustration of the ability of the cerebral angiogram to highlight the pathoanatomy of the sturgeweber syndrome sws, also known as encephalotrigeminal angiomatosis. The first page of the pdf of this article appears above. Fuchs 1882 published the description and drawings of the classical red peripheral tumor with the typically dilated artery and veins, but considered the lesion to be an arteriovenous aneurysm 23. Retinal angiomatosis has both a hereditary and a sporadic form. The condition is usually considered a variant of reactive angioendotheliomatosis. Angiomatosis encefalotrigeminal angiomatosis leptomeningea angiomatosis encfalocutanea angiomatosis meningocapilar angiomatosis meningofacial. This condition occurs when cells that line blood vessels grow into the surrounding skin tissue. We are open fo r joining other investigators who feel willing and possibilities to improve our.
Sturgeweber syndrome, also called encephalotrigeminal angiomatosis, is a rare brain disorder that causes seizures and other neurological symptoms, which may include glaucoma, learning disabilities, intellectual disabilities formerly called mental retardation, complete or partial paralysis of one side of the body, and muscle weakness. The patients commonly present with severe intractable breast pain and characteristic reticular violaceous erythematous plaques with central ulcerations. Bacillary angiomatosis is a vascular, proliferative form of bartonella infection that occurs primarily in immunocompromised persons. The expression of the syndrome is variable1 but typically manifests as facial portwine stain pws, ocular choroidal hemangiomas, and cerebral pial angiomatosis. Diffuse dermal angiomatosis of the breast allergy and. Diffuse dermal angiomatosis dda is a benign, acquired, reactive vascular proliferation. On the other hand, bartonella quintana is usually transmitted by lice. Skeletal angiomatosis rare cause of bone destruction. Pathology outlines hemangioma and angiomatosis of breast. Venous malformations and arteriovenous malformations used to be synonyms of angiomatosis previously, but have now been redefined. Cystic angiomatosis is a rare entity characterized by hamartomatous malformation of primitive vessels.
It affects patients from infancy to the sixth decade but typically occurs in young women. Diffuse cerebral angiomatosis is a rare disorder of the brain and only 5 cases have been reported to date. Symptoms may include headaches, problems with balance and walking, dizziness, weakness. In the series by rao and weiss 2,which included 51 cases of soft tissue angiomatosis, the oldest patient was 54 years, and more than half of the cases occurred in the lower extremities. Angiomatosis is a nonneoplastic condition 1 presenting with little knots of capillaries in various organs. Sws is part of a family of neurocutaneous disorders, characterized by abnormalities of ecto.
If angiomatosis occurs in the brain, then depending on the size and location, the prognosis will vary from excellent to possible death. Diffuse cutaneous angiomatosis is one of the reactive angiomatoses. It is characterized histologically by a diffuse proliferation of endothelial cells arranged singly in the interstitial spaces between collagen bundles throughout. Diffuse dermal angiomatosis 1 is a rare complication of ischemia and may be secondary to atherosclerosis, 2 anterior venous fistula, 3 or macromastia. Angiomatosis usually presents in the first two decades of life mostly in childhood or adolescence with a slight predilection for females. Sometimes these purple patches can become open wounds in the skin ulcerations, which may be painful. The disease is only rarely seen in healthy immunocompetent. Angiomatosis is a nonneoplastic condition characterised by nests of proliferating capillaries arranged in a lobular pattern, displacing adjacent muscle and fat. Sturgeweber syndrome sws is a neurocutaneous disorder characterized by angiomatosis of the skin, eye, and meninges. A 48yearold woman presented for routine dental treatment.
Meningioangiomatosis ma is a rare, benign lesion that commonly mimics other intracranial malformations in clinical presentation and appearance on imaging. Bacillary angiomatosis presenting with facial tumor and. It was first described in 1983 in a patient infected with the human immunodeficiency virus hiv. The first reports on retinal angiomatosis are attributed to magnus 1874, jackson 1878, and panas and remy 1879 58, 43, 65. Diffuse dermal angiomatosis is a rare condition in which purplish patches develop in the skin, most often on the legs, though they may occur in other areas of the body. Browse our catalog of research reagents for congenital leptomeningeal angiomatosis including antibodies and elisa kits against tie1, pdgfra, cd34, vwf, flk1. Bartonella henselae is most often transmitted through a cat scratch or bite, though ticks and fleas may also act as a vector. A cause of unbearably painful breast, diffuse dermal angiomatosis diffuse dermal angiomatosis is a rare disorder, considered to be a variant of reactive cutaneous angioendotheliomatosis. Bacillary angiomatosis is a systemic illness characterised by lesions similar to those of kaposi sarcoma in the skin, mucosal surfaces, liver, spleen and other organs. Sturgeweber syndrome sws, or encephalotrigeminal angiomatosis, is a rare, congenital neurocutaneous syndrome characterized by unilateral facial cutaneous vascular malformation nevus flammeus or portwine stain pws in association. The retinal andor optic disc lesions that are limited to the eye have. Angiomatosis diffuse hemangioma of the breast is a rare benign entity. The mode of transmission is autosomal dominant, often with incomplete penetrance and variable expression.
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