Angiokeratoma Circumscriptum Workup
- Author: William P Baugh, MD; Chief Editor: William D James, MD more...
No laboratory studies are required to identify or diagnose this entity.
Imaging studies are not usually indicated in the evaluation of this superficial cutaneous vascular lesion. If multiple grouped angiokeratomas are found overlying the spine in a newborn or an infant, an MRI of the spine may be prudent to exclude spinal dysraphism or Cobb syndrome.
The laboratory evaluation that confirms the diagnosis is a biopsy. Depending upon the size, shape, and estimated depth of the lesion, a shave or punch biopsy is usually performed. The biopsy result will eliminate melanoma from the clinical differential.
The histopathologic features of angiokeratoma circumscriptum are similar to those seen in other clinical types of angiokeratomas (eg, Mibelli type, Fordyce type, Fabry disease). The process has an exophytic profile, with numerous ectatic thin-walled vascular channels that expand the papillary dermis. Thrombosis of these vessels is common and is responsible for the clinical mimicry of melanoma. The overlying epidermis encompasses the vascular spaces, often with a collarette, and displays variable degrees of acanthosis and hyperkeratosis. The hyperkeratotic scale may be orthokeratotic and parakeratotic. The dermal connective tissue is usually not involved, but it may contain a few siderophages.
The lesions of Fabry disease may be differentiated from other forms of angiokeratoma because lipid-containing cytoplasmic vacuoles can sometimes be detected in endothelial cells, fibroblasts, and pericytes.
Lymphangioma circumscriptum, perhaps the most similar to angiokeratoma circumscriptum, is a clinically distinctive vascular malformation consisting of dilated lymphatic channels arrayed within the papillary dermis. Sometimes, these lymphangiectatic spaces are filled with serosanguineous lymph fluid, but hemorrhage into the spaces can render them microscopically indistinguishable from those of angiokeratomas. At present, no immunoperoxidase markers allow definitive distinction of blood vascular endothelium from lymphatic vascular endothelium.
Verrucous hemangioma is a descriptive term that is used to classify conventional hemangiomas in which associated verrucous epidermal changes are present. Although the superficial changes in a verrucous hemangioma can be identical to those of an angiokeratoma, usually a greater degree of depth and vascular proliferation are evident in the hemangioma. In short, angiokeratomas are typically confined to the papillary dermis, whereas verrucous hemangiomas involve all levels of the dermis and may extend to involve the subcutis.
Other vascular lesions can be associated with capillary dilatation. The diverse list of diseases includes entities such as generalized essential telangiectasia, unilateral nevoid telangiectasia, angioma serpiginosa, and Osler-Weber-Rendu disease. These syndromes lack the overlying epidermal changes seen in angiokeratomas; therefore, they are not usually included in the pathologic differential diagnosis of angiokeratoma.
Ozdemir R, Karaaslan O, Tiftikcioglu YO, Kocer U. Angiokeratoma circumscriptum. Dermatol Surg. 2004 Oct. 30(10):1364-6. [Medline].
Feramisco JD, Fournier JB, Zedek DC, Venna SS. Eruptive angiokeratomas on the glans penis. Dermatol Online J. 2009. 15(10):14. [Medline].
Eskiizmir G, Gencoglan G, Temiz P, Ermertcan AT. Angiokeratoma circumscriptum of the tongue. Cutan Ocul Toxicol. 2011 Sep. 30(3):231-3. [Medline].
Somasundaram V, Premalatha S, Rao NR, Razack EM, Zahra A. Hemangiectatic hypertrophy with angiokeratoma circumscriptum. Int J Dermatol. 1988 Jan-Feb. 27(1):45-6. [Medline].
Sodaifi M, Aghaei S, Monabati A. Cutaneous variant of angiokeratoma corporis diffusum associated with angiokeratoma circumscriptum. Dermatol Online J. 2004 Jul 15. 10(1):20. [Medline].
Sardana K, Koranne RV, Sharma RC, Mahajan S. Angiokeratoma circumscriptum naeviforme: rare presentation on the neck. Australas J Dermatol. 2001 Nov. 42(4):294-5. [Medline].
Ghosh SK, Bandyopadhyay D, Ghoshal L, Haldar S. Angiokeratoma circumscriptum naeviforme: a case report of a rare disease. Dermatol Online J. 2011 Sep 15. 17(9):11. [Medline].
Imperial R, Helwig EB. Verrucous hemangioma. A clinicopathologic study of 21 cases. Arch Dermatol. 1967 Sep. 96(3):247-53. [Medline].
Schiller PI, Itin PH. Angiokeratomas: an update. Dermatology. 1996. 193(4):275-82. [Medline].
Lynch PJ, Kosanovich M. Angiokeratoma circumscriptum. Arch Dermatol. 1967 Dec. 96(6):665-8. [Medline].
Bechara FG, Happle R, Altmeyer P, Grabbe S, Jansen T. Angiokeratoma circumscriptum arranged in a systematized band-like pattern suggesting mosaicism. J Dermatol. 2006 Jul. 33(7):489-91. [Medline].
Yildirim M, Kilinc N, Oktay MF, Topcu I. A case of solitary angiokeratoma circumscriptum of the tongue. Kulak Burun Bogaz Ihtis Derg. 2007. 17(6):333-5. [Medline].
Kumar MV, Thappa DM, Shanmugam S, Ratnakar C. Angiokeratoma circumscriptum of the oral cavity. Acta Derm Venereol. 1998 Nov. 78(6):472. [Medline].
Goldman L, Gibson SH, Richfield DF. Thrombotic angiokeratoma circumscriptum simulating melanoma. Arch Dermatol. 1981 Mar. 117(3):138-9. [Medline].
Foucar E, Mason WV. Angiokeratoma circumscriptum following damage to underlying vasculature. Arch Dermatol. 1986 Mar. 122(3):245-6. [Medline].
Ilyas EN, Seykora JT, Heymann WR. Acquired agminated acral angioma: a novel vascular lesion. Arch Dermatol. 2005 May. 141(5):646-7. [Medline].
Rossi A, Bozzi M, Barra E. Verrucous hemangioma and angiokeratoma circumscriptum: clinical and histologic differential characteristics. J Dermatol Surg Oncol. 1989 Jan. 15(1):88-91. [Medline].
Wang G, Li C, Gao T. Verrucous hemangioma. Int J Dermatol. 2004 Oct. 43(10):745-6. [Medline].
Occella C, Bleidl D, Rampini P, Schiazza L, Rampini E. Argon laser treatment of cutaneous multiple angiokeratomas. Dermatol Surg. 1995 Feb. 21(2):170-2. [Medline].
Pasyk KA, Argenta LC, Schelbert EB. Angiokeratoma circumscriptum: successful treatment with the argon laser. Ann Plast Surg. 1988 Feb. 20(2):183-90. [Medline].
Gorse SJ, James W, Murison MS. Successful treatment of angiokeratoma with potassium tritanyl phosphate laser. Br J Dermatol. 2004 Mar. 150(3):620-2. [Medline].
del Pozo J, Fonseca E. Angiokeratoma circumscriptum naeviforme: successful treatment with carbon-dioxide laser vaporization. Dermatol Surg. 2005 Feb. 31(2):232-6. [Medline].