Angiokeratoma circumscriptum D23.L

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 05.08.2021

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angiokeratoma circumscriptum; angiokeratoma circumscriptum naeviforme; Angiokeratoma corporis circumscriptum naeviforme; angiokeratoma corporis naeviforme; angiokeratoma naeviforme; angioma verrucosum; Blood vessel nevi; Hemangioma verrucous; verrucous haemangioma

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Fabry, 1915

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Not quite rare, unilateral, mostly monotopic, gynecotropic, venous malformation mainly of the lower extremity.

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Mostly congenital, rarely occurring during childhood. Women are 3 times more frequently affected than men.

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Lower extremities, rare strain.

Clinical features
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Single or plural, isolated or confluent, light red to violet-red, but in places also blue to black-red or black (thrombosed), flat but also sour, clearly consistency increased plaques or nodules.

Single plaques usually only a few centimetres in size; confluent plaques up to the size of the palm of the hand, possibly affecting an entire limb; also possible in a linear arrangement. Usually no subjective complaints.

Local accidental bleeding possible.

Commonly occurring as a sign of complex venous malformation in combination with nevus flammeus, veinctasia and osteohypertrophy.

S.a.u. Cobb syndrome.

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Hyperkeratosis, papillomatosis, irregular acanthosis. Volumes of strongly dilated, erythrocyte-filled capillaries throughout the corium, sometimes extending into the subcutis.

Various authors refer to the angiokeratoma circumscriptum with vascular parts reaching the deep tissue layers including subcutis and fascia as verrucous hemangioma.

Differential diagnosis
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With flat angiokeratomas, an experiment with laser therapy ( argon laser) is possible. For verrucous forms excision in LA.

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Initially mostly flat, well compressible, red-blue plaque; over the years proportional size growth; at the same time thickness growth with a verrucous surface. No regression tendency. Not infrequently, surface bleeding after banal injuries and thrombosis so that the colour can change from red-blue to homogeneous black (DD: malignant melanoma).

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  1. Anderson W (1898) A case of "angio-keratoma". Br J Dermatol (Oxford) 10: 113-117
  2. del Pozo J, Fonseca E (2005) Angiokeratoma circumscriptum naeviforme: successful treatment with carbon-dioxide laser vaporization. Dermatol Surg 31: 232-236
  3. Fabry J (1915) On a case of angiokeratoma circumscriptum of the left thigh. Dermatological Journal 22: 1-4
  4. Fabry J (1916) On the clinic and etiology of angiokeratoma. Archiv für Dermatologie und Syphilis (Berlin) 123: 294-307.
  5. Occella C et al (1995) Argon laser treatment of cutaneous multiple angiokeratomas. Dermatol Surg 21: 170-172

  6. Sadana D et al (2014) Angiokeratoma circumscriptum in a young male. Indian J Dermatol 59:85-87
  7. Vijaikumar M et al (2003) Angiokeratoma circumscriptum of the tongue. Pediatr Dermatol 20: 180-182
  8. Wang Let al (2014) Verrucous hemangioma: a clinicopathological and immunohistochemical analysis of 74 cases. J Cutan Pathol 41:823-830.


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Last updated on: 05.08.2021