Angiokeratoma Circumscriptum

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.

This disorder stems from the following process: congenital.


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].

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    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].


    Other Pathologies
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    • Mibelli type: The "Mibelli-type" occurs on the acral sites, mainly digits, of young people affected by repeated attacks of chilblain, which result in a deleterious effects on vessel walls. 2.[]
    • Angiokeratoma of Mibelli has been associated with acrocyanosis and chilblains.[]
    • Angiokeratoma of Mibelli has been associated with acrocyanosis and chilblains[]
    • […] has been speculated that the increased cell proliferation on the surface of vascular malformations and the close location of the vascular spaces with the epidermis in angiokeratoma could explain the reactive epidermal growth. [1] Pregnancy, trauma, chilblains[]
    • Localized angiokeratoma may be seen in a setting of acute or chronic trauma, chilblains, high venous pressure, or nevoid or vascular malformation.[]
    • […] circumscriptum, angiokeratoma corporis diffusum, and angiokeratoma of fordyce. [1] , [2] Angiokeratoma of fordyce involves the scrotum or vulva usually of the middle-aged or elderly individuals, but may also arise in the second or third decade. [2] Urethral[]
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    Oral Contraceptive
    • The other potential precipitants are intra-abdominal masses, urinary tract tumors, and varicoceles. [4] Vulvar lesions may be associated with vulvar varicosities, oral contraceptive pills, hemorrhoids, hysterectomy, or increased venous pressure during[]
    • Angiokeratoma of Fordyce is a condition that most commonly affects elderly individuals, although similar lesions can occur in pregnant females or with the use of oral contraceptive pills.[]


    Sex distribution
    Age distribution




    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.

    Patient Information

    Other symptoms

    • Comments Late onset, unusual site, bilateral involvement, intermittent bleeding and associated extensive telangiectasia are certain unusual features observed in this case.[]
    • It presents as a warty, bluish-purple plaque, with intermittent oozing or bleeding.[]
    • Hemangiomas were present beneath the lesions in three cases, and in two patients lymphangioma circumscripta was present between hemangiomas, indicating an intermediate form of angiokeratoma circumscriptum.[]
    Angiokeratomas of Soles
    • Till date there are three reported cases of angiokeratoma circumscriptum solely localized to the oral cavity in pediatric patients [2-4].[]


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    1. 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.
    2. Happle R. Capillary malformations: a classification using specific names for specific skin disorders. J Eur Acad Dermatol Venereol. 2015; 29(12):2295-2305.
    3. Imperial R, Helwig EB. Angiokeratoma. A clinicopathological study. Arch Dermatol. 1967; 95(2):166-175.
    4. 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.
    5. Schiffmann R, Ries M. Fabry Disease open link: A Disorder of Childhood Onset. Pediatr Neurol. 2016.
    6. Patrizi A, Neri I, Trevisi P, Landi C, Bardazzi F. Congenital angiokeratoma of Fordyce. J Eur Acad Dermatol Venereol. 1998; 10(2):195-196.
    7. 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.
    8. 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.
    9. 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.
    10. Aggarwal K, Jain VK, Jangra S, Wadhera R. Angiokeratoma circumscriptum of the tongue. Indian Pediatr. 2012; 49(4):316-318.
    11. Ozdemir R, Karaaslan O, Tiftikcioglu YO, Kocer U. Angiokeratoma circumscriptum. Dermatol Surg. 2004; 30(10):1364-1366.

    • 6 The Diagnosis - A Fissuratum, A Nigricans - Exp Dermatol - Springer
    • A case of angiokeratoma - H Vala, F Esteves - Veterinary Pathology Online, 2001 -


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