Angiokeratoma circumscriptum D23.L

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

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

History
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Fabry, 1915

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

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

Localization
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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.

Histology
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Hyperkeratosis, papillomatosis, irregular acanthosis. Convolutes of strongly dilated, erythrocyte-filled capillaries in the entire corium, sometimes extending into the subcutis.

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

Progression/forecast
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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).

Literature
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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 Surgery 31: 232-236
  3. Fabry J (1915) On a case of angiokeratoma circumscriptum on the left thigh. Dermatological journal 22: 1-4
  4. Fabry J (1916) On the clinic and etiology of angiokeratoma. Archive for Dermatology and Syphilis (Berlin) 123: 294-307
  5. Occella C et al (1995) Argon laser treatment of cutaneous multiple angiokeratomas. Dermatol Surgery 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. Pediatric dermatol 20: 180-182

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020