Angiokeratoma circumscriptum D23.L

Fabry, 1915

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

The mechanism for the development of angiokeratoma circumscriptum is unknown. It is speculated that altered hemodynamics, chronic trauma, tissue asphyxiation, congenital causes, pregnancy or frostbite play a role in the development of ectatic blood vessels in the papillary dermis below the basement membrane. The epidermal changes are reactive, increase with age and follow vascular ectasia. The condition is benign and is not usually associated with systemic disease. However, it has rarely been associated with Klippel-Trenauney syndrome and Cobbs syndrome.

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

Lower extremities, rare strain.

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.

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.

Initially mostly flat, easily compressible, red-blue plaque; proportional growth in size over the years; simultaneous growth in thickness with a verrucous surface. No tendency to regress. Not infrequently surface bleeding after banal injuries, also thrombosis, so that the color can change from red-blue to homogeneous black (DD: malignant melanoma).

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 on the left thigh. Dermatologic Journal 22: 1-4
4. Fabry J (1916) On the clinic and etiology of angiokeratoma. Archiv für Dermatologie und Syphilis (Berlin) 123: 294-307
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8. Vijaikumar M et al (2003) Angiokeratoma circumscriptum of the tongue. Pediatr Dermatol 20: 180-182
9. Wang Let al. (2014) Verrucous hemangioma: a clinicopathological and immunohistochemical analysis of 74 cases. J Cutan Pathol 41:823-830.