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Angiokeratoma circumscriptumD23.L
Synonym(s)
HistoryThis section has been translated automatically.
Fabry, 1915
DefinitionThis section has been translated automatically.
Not quite rare, unilateral, mostly monotopic, gynecotropic, venous malformation mainly of the lower extremity.
PathophysiologyThis section has been translated automatically.
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.
ManifestationThis section has been translated automatically.
Mostly congenital, rarely occurring during childhood. Women are 3 times more frequently affected than men.
LocalizationThis section has been translated automatically.
Lower extremities, rare strain.
ClinicThis section has been translated automatically.
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.
HistologyThis section has been translated automatically.
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 diagnosisThis section has been translated automatically.
TherapyThis section has been translated automatically.
Progression/forecastThis section has been translated automatically.
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).
LiteratureThis section has been translated automatically.
- Anderson W (1898) A case of "angio-keratoma". Br J Dermatol (Oxford) 10: 113-117
- del Pozo J, Fonseca E (2005) Angiokeratoma circumscriptum naeviforme: successful treatment with carbon-dioxide laser vaporization. Dermatol Surg 31: 232-236
- Fabry J (1915) On a case of angiokeratoma circumscriptum on the left thigh. Dermatologic Journal 22: 1-4
- Fabry J (1916) On the clinic and etiology of angiokeratoma. Archives of Dermatology and Syphilis (Berlin) 123: 294-307
Grewal AK et al. (2018) A case report of adult-onset multiple angiokeratomas with zosteriform distribution. Dermatol Online J 24:13030/qt9644290r.
- Ivy H et al (2024) Angiokeratoma Circumscriptum. 2023 Jul 17. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing 2024 PMID: 31747176.
- Occella C et al. (1995) Argon laser treatment of cutaneous multiple angiokeratomas. Dermatol Surg 21: 170-172
- Sadana D et al. (2014) Angiokeratoma circumscriptum in a young male. Indian J Dermatol 59:85-87
- Vijaikumar M et al (2003) Angiokeratoma circumscriptum of the tongue. Pediatr Dermatol 20: 180-182
- Wang Let al. (2014) Verrucous hemangioma: a clinicopathological and immunohistochemical analysis of 74 cases. J Cutan Pathol 41:823-830.