Angiokeratoma circumscriptum is a cutaneous lesion of dark red or reddish blue color. It is a benign vascular disorder that doesn't require any special treatment, but is usually resected for cosmetic reasons and because malignancies may be misdiagnosed as angiokeratomas.
Presentation
AC may be present at birth or develop at a later point in time [7]. Patients more often present with unilateral, solitary lesions than with bilateral disease or multiple AC. Besides cutaneous alterations as described below, the vast majority of AC patients remains asymptomatic. However, they may claim tenderness [8] [9].
AC are usually of dark red or reddish blue color, much like hematomas, but are raised above the skin surface. They are prone to bleed, and intradermal hemorrhages may cause the lesion to change color rapidly. AC appear as well-demarcated papules or plaques that frequently have irregular borders and a verrucous surface. In the case of multiple lesions, band-like patterns, and linear arrangements prevail. Most commonly, AC affect the distal portions of either lower limb, the thighs or buttocks. However, these angiokeratomas may develop virtually anywhere. Additionally, AC of the tongue and buccal mucosa have repeatedly been described [10].
Entire Body System
- Surgical Procedure
Treatment options for angiokeratomas Laser Therapy Surgical Procedures Local ablation Nd:YAG Excision Cryotherapy PDL Sclerotherapy Electrocautery CO2 Copper vapor Argon KTP PDL Optimal Therapeutic Approach for Angiokeratomas Mibelli and Fordyce Since [dermatologyadvisor.com]
Keywords: Ambulatory surgical procedures; Dermatology; Vascular malformations INTRODUCTION Angiokeratomas are relatively rare vascular malformations characterized by asymptomatic skin lesions. [surgicalcosmetic.org.br]
In 13 cases, the surgical procedure, either vulvectomy, 50 laser vaporization, 73 or excision of the lesions 7, 24, 43, 74 – 76 was conducted before any medical treatment, and a vulvar adenocarcinoma was found in one of these patients. [academic.oup.com]
- Congestive Heart Failure
Heart Failure Aysegul Senturk, Aysegul Karalezli, Ayse Nur Soyturk, H Canan Hasanoglu DOI :10.4103/2156-7514.115762 PMID :24083067 J Clin Imaging Sci 2013, 3:30 (29 July 2013) Transjugular Intrahepatic Portosystemic Shunt for Maintenance of Portal Venous [clinicalimagingscience.org]
- Turkish
Our study was presented for this rare condition in a Turkish male. KEY WORDS: Angiokeratoma - Tongue - Actinomycosis top of page [minervamedica.it]
- Malaise
Dyshidrotic Eczema (Pompholyx) cough sore throat rhinorrhea conjunctivitis malaise) then rash: painful. Can I take orally and topically for toddler rash benadryl recall april Cholestasis of pregnancy does help dyshidrotic eczema benadryl use long. [edenbio.eu]
Cardiovascular
- Heart Failure
Failure Treatment study.CONCLUSIONS: Increased UACR is common in patients with heart failure, including non-diabetics. [read.qxmd.com]
heart failure, strokes, arthritis, colitis and many other problems. [dermnetnz.org]
This leads to widespread cutaneous presentation, in addition to systemic symptoms such as eye disorders, kidney or heart failure, stroke, and arthritis. [ozarkderm.com]
Skin
- Papule
Multiple, well-defined, erythematous papules were present discretely over the abdomen, scrotum and the thighs. [jewd.eg.net]
[…] named after Italian dermatologist Vittorio Mibelli (1860-1910).[6] Angiokeratoma of Fordyce (also known as "Angiokeratoma of the scrotum and vulva," though not to be confused with Fordyce's spots)[5] is a skin condition characterized by red to blue papules [en.wikipedia.org]
[…] page) Answer: Angiokeratoma Angiokeratoma refers to a benign vascular telangiectasia in the papillary dermis that is associated with hyperkeratosis of the epidermis.1 The disorder is characterized clinically by solitary or multiple dark red or black papule [consultant360.com]
It usually presents as multiple,red, blue or black asymptomatic papules on lower extremities. Oral involvement, common in systemic form, is rare in localized forms. [unboundmedicine.com]
Neurologic
- Irritability
Most lesions are asymptomatic, but irritation, pain, a burning sensation, and itching occasionally may occur.2,5 Solitary angiokeratoma is the most common variant and accounts for 70% to 83% of cases.2,5 The condition was first described by Imperial and [consultant360.com]
These lesions likely won't have any irritating symptoms when they first form, but over time, they can become itchy and painful. You should talk to your doctor if you begin to feel any irritation from your angiokeratomas. [webmd.com]
Predisposing factors include mechanical trauma and chronic irritation. [oatext.com]
Provides intense and lasting relief for severely dry itchy or irritated skin. [edenbio.eu]
Intermittent bleeding following irritation of the lesions Is seen in 25 percent cases only. [1] Its association has been reported with naevus flammeus, [2] cavernous haemangioma, [3] Klippel-Trιnaunay syndrome, [6] angioma of spinal cord (Cobb syndrome [ijdvl.com]
Workup
It is of utmost importance to distinguish AC from malignant melanoma and other skin tumors [8] [11]. Histopathological analyses of biopsy specimens may strongly support the clinical diagnosis. Characteristic findings are [4]:
- Hyperkeratosis (orthokeratosis or parakeratosis)
- Acanthosis
- Papillomatosis
- Thin-walled, dilated capillaries in the papillary dermis
- Occasionally, vascular alterations comprise the reticular dermis
- Thrombosis of ecstatic vessels
AC have been shown to stain positive for lymphatic markers such as podoplanin and Prox1 [4].
Additional studies, namely biochemical and molecular biological analyses, may be indicated to rule out Fabry disease [5].
Treatment
[…] of angiokeratoma circumscriptum naeviforme with long pulse alexandrite laser. 62 Lorgeou A...Passeron T 27511129 2017 13 Extensive angiokeratoma circumscriptum - successful treatment with 595-nm variable-pulse pulsed dye laser and 755-nm long-pulse pulsed [malacards.org]
Treatment Options Treatment options are summarized in Table I. Table I. [dermatologyadvisor.com]
We recommend VPPDL and LPPAL for the treatment of extensive AC. [ncbi.nlm.nih.gov]
Argon laser treatment of cutaneous multiple angiokeratomas. Dermatol Surg. 1995 Feb. 21(2):170-2. [QxMD MEDLINE Link]. Pasyk KA, Argenta LC, Schelbert EB. Angiokeratoma circumscriptum: successful treatment with the argon laser. [emedicine.medscape.com]
Prognosis
The treatment options include removal of the tumor through surgery The prognosis of Verrucous Hemangioma is good with suitable treatment. [dovemed.com]
Etiology
Lu Angiokeratoma circumscriptum vascular lesions are defined by ectasia of the papillary dermal vessels, and the etiology is unknown. [go.gale.com]
We describe the clinical and histopathological findings in a patient with angiokeratoma circumscriptum and discuss the etiology, associations, diagnosis, differential diagnosis, and treatment. [escholarship.org]
Klippel-Trenaunay syndrome: diagnostic criteria and hypothesis on etiology. Ann Plast Surg. 2008;60(2):217–23. Jindal SR, Chalvade P, Jerajani HR. Late onset palmar angiokeratoma circumscriptum: An unusual presentation. [ijord.com]
Dermatological Journal 22: 1-4 Fabry J (1916) On the clinic and etiology of angiokeratoma. Archiv für Dermatologie und Syphilis (Berlin) 123: 294-307. Occella C et al (1995) Argon laser treatment of cutaneous multiple angiokeratomas. [altmeyers.org]
Epidemiology
Topics covered includes: Epidemiology of Contact Dermatitis, Animal Models of Contact Dermatitis, Keratinocytes, Innate Immunity and Allergic Contact Dermatitis, Topical Delivery of Haptens: Methods of Modulation of the Cutaneous Permeability to Increase [freebookcentre.net]
Overall, altered hemodynamics (typically caused by trauma) appear to produce telangiectatic vessels of the papillary dermis with an overlying reactive hyperkeratosis to the epidermis. [9] Epidemiology Frequency United StatesThe frequency of angiokeratoma [thehealthscience.com]
♦ Skin testing ♦ Radiological and imaging ♦ Commonly used laboratory tests examination ♦ Oral provocation test EPIDEMIOLOGY OF SKIN DISEASE Must know Should know Good to know ♦ What is epidemiology and why is it relevant to dermatology ♦ Describing [medicaldialogues.in]
Epidemiology Frequency The frequency of angiokeratoma circumscriptum in the United States is unknown, yet it has been reported worldwide to occur in 16% of the population. [emedicine.medscape.com]
EPIDEMIOLOGY The prevalence of angiokeratoma is estimated to be approximately 0.16% among the general population.2,5 The disorder is most prevalent in the white population and is less commonly observed in dark-skinned populations.2 The peak age of onset [consultant360.com]
Pathophysiology
Pathophysiology[edit] Histology[edit] Angiokeratomas characteristically have large dilated blood vessels in the superficial dermis and hyperkeratosis (overlying the dilated vessels). [en.wikipedia.org]
Several causal factors, such as congenital development, pregnancy, trauma, [12] subcutaneous hematomas, and tissue asphyxia, have all been proposed (see Pathophysiology). [emedicine.medscape.com]
Angiokeratoma circumscriptum has also been called angiokeratoma corporis naeviform and may be best classified as a type of capillary malformation. [6, 7] Pathophysiology As Imperial and Helwig discussed in 1967, angiokeratomas are not true angiomas but [thehealthscience.com]
The pathophysiology of the lesion is discussed with a review of the pertinent literature. It appears that LC primarily affects the subcutaneous tissue in the form of cystic dilatation of lymphatic channels without systemic lymphatic communication. [link.springer.com]
Pathophysiology Histology Angiokeratomas characteristically have large dilated blood vessels in the superficial dermis and hyperkeratosis (overlying the dilated vessels). [foxhold.com]
Prevention
Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets [books.google.com]
To learn more or modify/prevent the use of cookies, see our Cookie Policy and Privacy Policy. Angiokeratomas are papular telangiectasias having a common histology of ectasia of the superficial dermal vessels surmounted by a hyperkeratotic epidermis. [researchgate.net]
But, many are known to reappear following their removal Please find comprehensive information on Verrucous Hemangioma regarding definition, distribution, risk factors, causes, signs & symptoms, diagnosis, complications, treatment, prevention, prognosis [dovemed.com]
Summary
Angiokeratomas are benign cutaneous lesions characterized by ectasia of papillary dermis vessels and secondary epidermal changes like hyperkeratosis [1]. It is important to note that they are not neoplasms [2]. Patients may present with solitary or multiple angiokeratomas that develop in distinct parts of the skin, differ in size and surface appearance. Accordingly, the following subtypes are distinguished [3] [4]:
- Angiokeratoma circumscriptum (AC), either solitary or arranged in a band-like pattern, most commonly affecting the legs
- Angiokeratoma corporis diffusum, multiple pinpoint lesions seen in patients suffering from Fabry disease, a lysosomal storage disorder [5]
- Angiokeratoma of Mibelli with acral localization
- Angiokeratoma of Fordyce affecting scrotum, penis or vulva [6]
- Solitary or multiple angiokeratomas
AC is the least common type of angiokeratoma and may be associated with Klippel-Trenaunay-Weber syndrome, Cobb syndrome, and other vascular malformations. Women are affected significantly more often than men. Contrary to untreated Fabry disease and malignancies of the skin, this disease follows a benign course and treatment is generally not required. The latter does not apply, however, if doubts remain as to the nature of the papule or plaque, or if patients request therapy for cosmetic reasons. Treatment may comprise the surgical excision of the respective lesions, but this approach may not be feasible in case of extensive AC. Laser therapy is increasingly applied in those cases.
References
- Lorgeou A, Chiaverini C, Le Duff F, Lacour JP, Passeron T. Successful treatment of angiokeratoma circumscriptum naeviforme with long pulse alexandrite laser. J Eur Acad Dermatol Venereol. 2016.
- Happle R. Capillary malformations: a classification using specific names for specific skin disorders. J Eur Acad Dermatol Venereol. 2015; 29(12):2295-2305.
- Imperial R, Helwig EB. Angiokeratoma. A clinicopathological study. Arch Dermatol. 1967; 95(2):166-175.
- Trindade F, Torrelo A, Kutzner H, Requena L, Tellechea O, Colmenero I. An immunohistochemical study of angiokeratomas of children. Am J Dermatopathol. 2014; 36(10):796-799.
- Schiffmann R, Ries M. Fabry Disease open link: A Disorder of Childhood Onset. Pediatr Neurol. 2016.
- Patrizi A, Neri I, Trevisi P, Landi C, Bardazzi F. Congenital angiokeratoma of Fordyce. J Eur Acad Dermatol Venereol. 1998; 10(2):195-196.
- Das A, Mondal AK, Saha A, Chowdhury SN, Gharami RC. Angiokeratoma circumscriptum neviforme: An entity, few and far between. Indian Dermatol Online J. 2014; 5(4):472-474.
- Dumitrache N, Neiderer K, Martin B, Dancho J. Angiokeratoma presenting as a melanoma: a case report. J Am Podiatr Med Assoc. 2013; 103(3):241-242.
- Sadana D, Sharma YK, Dash K, Chaudhari ND, Dharwadkar AA, Dogra BB. Angiokeratoma circumscriptum in a young male. Indian J Dermatol. 2014; 59(1):85-87.
- Aggarwal K, Jain VK, Jangra S, Wadhera R. Angiokeratoma circumscriptum of the tongue. Indian Pediatr. 2012; 49(4):316-318.
- Ozdemir R, Karaaslan O, Tiftikcioglu YO, Kocer U. Angiokeratoma circumscriptum. Dermatol Surg. 2004; 30(10):1364-1366.