DiseasesChemicals and Drugs
Angiokeratomaalpha-N-AcetylgalactosaminidaseFabry DiseaseTongue NeoplasmsSkin Neoplasms
DiffusumLesionsBenignCircumscriptumHemangiomasLesionLymphangiomaSolitaryForms of angiokeratomaEndothelial cellsCauses angiokeratomasBirthmarksPapulesFabryVulvaBirthmark CenterCutaneous vascularCapillaryMalformationsDiagnosisAcroparesthesiasSkinEndotheliumDermisVulvarDermatologistMalignantAnomaliesRenalSpotsKidney
Diffusum6
  • Angiokeratoma corporis diffusum is the cutaneous hallmark of Fabry disease, an X-linked inherited disorder caused by a deficiency in the lysosomal enzyme alpha-galactosidase A. Decreased or absent enzyme activity causes uncleaved glycosphingolipids to accumulate in various cell types, particularly in the vascular endothelium, smooth muscle cells, and pericytes, causing ischemia and infarction of tissues. (medscape.com)
  • Angiokeratoma corporis diffusum is not unique to Fabry disease and has also been documented in several other rare lysosomal storage disorders such as fucosidosis, sialidosis, GM1 gangliosidosis, galactosialidosis, beta-mannosidosis, Kanzaki disease, and aspartylglucosaminuria. (medscape.com)
  • Based on the clinical presentation and evolution, the current classification of angiokeratomas includes solitary and multiple angiokeratoma, angiokeratoma of Fordyce, angiokeratoma of Mibelli, angiokeratoma circumscriptum, and angiokeratoma corporis diffusum [ 1 , 2 , 3 ]. (cdlib.org)
  • Angiokeratoma corporis diffusum is a symptom of Farby syndrome - a rare but serious genetic condition. (contourderm.com)
  • Mornchan R. , Puvabanditsin P. Angiokeratoma Diffusum (Fabry 's disease): A Case Report. (ishrs.org)
  • Type 2 is characterized by milder psychomotor retardation and neurologic signs, the development of angiokeratoma corporis diffusum, normal sweat salinity, and longer survival (Kousseff et al. (nih.gov)
Lesions17
  • The terminology of vascular conditions can be confusing, with several lesions being incorrectly named or classified. (dermnetnz.org)
  • Angiokeratomas are relatively rare vascular lesions characterized by ectasias of the papillary dermal blood vessels with secondary epidermal changes in the form of acanthosis and /or hyperkeratosis. (cdlib.org)
  • Angiokeratomas are relatively rare vascular lesions characterized by dilatations of the papillary dermal blood vessels with secondary epidermal changes of acanthosis and /or hyperkeratosis [ 1 ]. (cdlib.org)
  • Angiokeratomas are acquired vascular lesions that present as shiny, soft, dark red to violaceous, 2-10 mm papules which may develop a blue-violaceous to black color and a surface scale. (dermoscopedia.org)
  • Angiokeratomas are benign vascular skin lesions composed of dilated capillaries. (contourderm.com)
  • Angiokeratoma of Mibelli is most common in young females and typically appears as one or multiple lesions on the fingers and toes. (contourderm.com)
  • Angiokeratoma of Fordyce is more common in men and presents as multiple lesions on the scrotum or vulva. (contourderm.com)
  • From this group of disorder, angiokeratomas and lymphangiectasis are the lesions most frequently seen in the vulva . (natoursprung.de)
  • Clinically, angiokeratoma of Fordyce over the vulva should be differentiated from angiomas (cherry angiomas, pyogenic granuloma, and lymphangioma), verrucous lesions (seborrhoeic keratosis, condyloma acuminata, nodular prurigo, condyloma lata, and certain nevi), and malignancies (melanoma and vulvar intraepithelial neoplasia). (natoursprung.de)
  • Cobb syndrome is an exceedingly rare non inherited condition characterised by vascular cutaneous lesions in the same metamere with a spinal arteriovenous malformation (SAVM) or a spinal angioma [ 1-3 ]. (panafrican-med-journal.com)
  • Vascular lesions are a commonly encountered problem in procedural dermatology, and patients frequently desire their removal. (mhmedical.com)
  • This chapter will discuss the evolution of light-based therapies, the treatment of vascular lesions using the principles of photothermolysis, and will review diagnostic criteria and suggested treatments for many vascular lesions including port wine stain (PWS) birthmarks, infantile hemangiomas (IHs), venous malformations (VMs), telangiectasias and rosacea, cherry and spider angiomas, poikiloderma of Civatte, and angiokeratomas. (mhmedical.com)
  • One of the first indications for the use of lasers in dermatologic surgery was for the treatment of vascular lesions. (mhmedical.com)
  • It is thus absorbed by hemoglobin in the superficial vessels of vascular lesions. (mhmedical.com)
  • 3 Although the argon laser, and other continuous and quasi-continuous wave lasers such as the copper vapor, krypton-ion, argon-pumped dye, and carbon dioxide lasers, improved the color of the vascular lesions, they also caused a large amount of nonselective heating due to long pulse durations and unwanted absorption by epidermal melanin. (mhmedical.com)
  • These lasers are no longer used for treatment of vascular lesions, as the theory of selective photothermolysis revolutionized the use of lasers for treatment of skin lesions. (mhmedical.com)
  • Diagnosis in males is clinical, based on appearance of typical skin lesions (angiokeratomas) over the lower trunk and by characteristic features of peripheral neuropathy (causing recurrent burning pain in the extremities), corneal opacities, and recurrent febrile episodes. (msdmanuals.com)
Benign7
  • Angiomas are benign vascular tumors that have the dermoscopic features of lacunae and septae. (dermoscopedia.org)
  • Pyogenic granuloma (PG) is a relatively common benign acquired friable vascular growth of the skin and mucous membranes often started by trauma. (dermoscopedia.org)
  • Almost all angiokeratomas are benign and require no treatment. (contourderm.com)
  • Lobular capillary hemangioma , or pyogenic granuloma, is an acquired hemorrhagic benign vascular lesion of the skin and mucous membranes. (natoursprung.de)
  • Outcomes/Resolutions) Angiokeratoma of Vulva is a common, benign (non-cancerous) condition that has an excellent prognosis with appropriate treatment. (natoursprung.de)
  • Cherry hemangiomas (also known as cherry angiomas, Campbell de Morgan spots, and senile hemangiomas) are the most common type of acquired benign vascular proliferation and are composed of thin-walled, dilated capillaries. (logicalimages.com)
  • Hemangioma is a very common benign vascular tumour . (librepathology.org)
Circumscriptum7
  • Amongst the several variants of angiokeratomas, angiokeratoma circumscriptum is the least common one. (cdlib.org)
  • Angiokeratoma circumscriptum naeviforme (ACN) is a still rarer type of angiokeratoma, which is typically seen at birth. (cdlib.org)
  • Angiokeratoma circumscriptum, the rarest variant [ 1 , 3 ], is clinically characterized by dark red to blue-black nodules or plaques that are typically situated unilaterally on the lower legs or feet. (cdlib.org)
  • Angiokeratoma circumscriptum naeviforme (ACN) is a still rarer variant of angiokeratoma, classically present since birth [ 4 ]. (cdlib.org)
  • Based on the clinical and histopathological features, a diagnosis of angiokeratoma circumscriptum naeviforme was made. (cdlib.org)
  • Angiokeratoma circumscriptum is a birthmark with a higher incidence in females and may change in appearance over time. (contourderm.com)
  • Its main differential diagnosis is angiokeratoma circumscriptum neviforme, with an almost indistinguishable clinical presentation because both diseases appear as erythematous patches that evolve to violaceous plaques, becoming scaly and even verrucous, most commonly affecting the lower limbs. (ufrgs.br)
Hemangiomas2
  • Hemangiomas of infancy are the most common vascular tumors in infants. (nicklauschildrens.org)
  • Infantile hemangiomas are arrested in an early developmental vascular differentiation state. (librepathology.org)
Lesion5
  • Solitary angiokeratoma appears as a single lesion, most commonly on the legs of the middle aged and elderly. (contourderm.com)
  • Cobb syndrome is a rare metameric disorder, characterized by either an angioma or an arteriovenous malformation of the spinal cord, with an associated vascular skin lesion of the same metamere. (panafrican-med-journal.com)
  • He had a congenital cutaneous vascular back lesion covering the same dermatomes. (panafrican-med-journal.com)
  • Cobb syndrome is suspected when you get spinal neurological deficits in a patient with a congenital cutaneous vascular lesion [ 4 ]. (panafrican-med-journal.com)
  • Red and dark spots after lip filler injections can also be a vascular lesion caused by bleeding beneath the skin surface. (aestheticami.com)
Lymphangioma2
Solitary2
Forms of angiokeratoma2
Endothelial cells2
  • 4 The deposition of ceramide trihexoside in lysosomes is found predominantly in vascular endothelial cells, smooth muscle cells, cardiac myocytes, leukocytes, fibroblasts, and epithelial cells of ganglion, corneal, and renal origins. (hcplive.com)
  • The endothelial cells of the vascular channels do not have atypia. (librepathology.org)
Causes angiokeratomas2
  • This process in the cutaneous endothelium causes angiokeratomas. (medscape.com)
  • an inherited disorder of metabolism, caused by deficiency of alpha-galactosidase A, which causes angiokeratomas, acroparesthesias, corneal opacities, recurrent febrile episodes, and renal or heart failure. (msdmanuals.com)
Birthmarks3
  • Broken veins ( telangiectasia ) of the face and vascular birthmarks It can be unpleasant and difficult to hide. (doctorhoogstra.com)
  • Certain vascular birthmarks are typically more serious than others and pose a health concern. (birthmarkcare.com)
  • The specialist team at the Vascular Birthmark Center can provide advanced diagnosis and treatments when necessary for these and other types of birthmarks. (birthmarkcare.com)
Papules2
  • Angiokeratomas in Fabry disease typically appear in childhood or adolescence and present as small red-to-black papules that most commonly affect the bathing trunk area (buttocks, groin, umbilicus, upper thighs), although almost any area of the body can be involved. (medscape.com)
  • Purple, vascular papules on left labium major. (vulvovaginaldisorders.org)
Fabry2
  • A high index of suspicion for Fabry disease should be noted, especially when angiokeratomas are seen with other earlier symptoms of the disease (acroparesthesia, hypohidrosis, or heat intolerance). (medscape.com)
  • Later symptoms ▫ Progressive renal failure, cardiovascular disease, heat/cold intolerance, hypohidrosis (decreased sweat production), hearing loss DIAGNOSIS LAB RESULTS ▪ Enzymatic assay for alpha galactosidase ▪ Genetic testing TREATMENT OTHER INTERVENTIONS Figure 52.1 Numerous angiokeratomas on the skin of an individual with Fabry disease. (osmosis.org)
Vulva1
Birthmark Center1
  • If your baby's doctor suspects a need for further evaluation, you should contact us at The Vascular Birthmark Center in New York for an exam and consultation. (birthmarkcare.com)
Cutaneous vascular1
Capillary1
  • A paediatric dermatologist , paediatrician, vascular specialist or surgeon should assess significant infantile capillary malformations or infantile haemangiomas , especially when large, symptomatic (especially when ulcerated), located on the head and neck or close to eye, nose or mouth. (dermnetnz.org)
Malformations1
  • Verrucous hemangioma is a rare vascular skin disorder with an immune profile similar to vascular neoplasms, but with behavior and evolution of vascular malformations. (ufrgs.br)
Diagnosis1
  • Histopathology is crucial for the correct diagnosis: while in angiokeratoma the vascular alterations are limited to the papillary dermis, verrucous hemangioma extends deep into the dermis, reaching the subcutaneous tissue. (ufrgs.br)
Acroparesthesias1
  • 8,10,11 The clinical characteristics that manifest in women range from fever, myalgia, and generalized weakness to whorl-like corneal epithelial dystrophy, angiokeratomas, and acroparesthesias. (hcplive.com)
Skin4
  • The dermis contains two vascular networks that run parallel to the skin surface-one superficial and one deep plexus-which are connected by vertical communicating vessels. (wikipedia.org)
  • It is a thin loose covering of keratinizing skin with associated underlying eccrine (sweat) and sebaceous glands and a highly vascular stroma without underlying adipose tissue. (medscape.com)
  • pain, angiokeratomas (skin spots), eye abnormalities (vertical cornea). (carenity.co.uk)
  • The sections show hair-bearing skin with abundant small superficial vascular channels containing red blood cells. (librepathology.org)
Endothelium1
  • The most affected are the vascular endothelium and smooth muscle cells. (medscape.com)
Dermis2
  • Dilated vascular spaces containing erythrocytes in the papillary dermis. (cdlib.org)
  • No vascular ectasia was found in the reticular dermis or in the subcutis. (cdlib.org)
Vulvar2
  • We report the development of multiple vulvar angiokeratomas in a 40-year-old female after undergoing a perineoplasty successfully treated with electrocautery. (natoursprung.de)
  • Angiokeratoma - Darker purple to blue color, scale or crust, and location on the genitals (scrotal, vulvar) or lower extremities are suggestive of angiokeratoma. (logicalimages.com)
Dermatologist1
Malignant1
  • Low-grade malignant vascular tumor caused by human herpesvirus-8 (HHV-8) producing a vascular proliferation. (dermoscopedia.org)
Anomalies1
  • Vascular naevi or anomalies are present at birth or appear in early childhood. (dermnetnz.org)
Renal1
  • Death results from renal failure or cardiac or cerebral complications of hypertension or other vascular disease. (msdmanuals.com)
Spots2
  • Angiokeratomas are small dark red to purple raised spots and come in several types. (nicklauschildrens.org)
  • Simple vascular lasers can treat redness associated with old acne spots. (drdavinlim.com)
Kidney1
  • The more serious problems that the disease can involve are caused by heart, kidney and vascular disease (heart attack). (carenity.co.uk)