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Angiokeratoma in Child
Other Resources UpToDate PubMed

Angiokeratoma in Child

Contributors: Tracy Lu, Sarah Hocker DO, Susan Burgin MD
Other Resources UpToDate PubMed


Angiokeratomas are asymptomatic, benign vascular neoplasms that are characterized histologically by superficial dermal vascular ectasia with varying degrees of overlying epidermal hyperplasia and hyperkeratosis.

Five clinical types of angiokeratoma are recognized (refer to individual subtypes for more detailed information and images):
  • Solitary or multiple angiokeratoma – Bluish to black verrucous plaques or nodules that develop on the lower extremities of adults.
  • Angiokeratoma of Mibelli – Single or multiple, punctate, pinkish macule(s) arise on the dorsum of fingers and toes in adolescence. Over time, some lesions become dark blue-red, papular, hyperkeratotic, and even verrucous. Papules may form grape-like clusters as they evolve. Perniosis or acrocyanosis may be associated. Familial cases have been reported.
  • Angiokeratoma corporis diffusum – Multiple tiny red macules and papules are scattered predominantly on the lower torso and thighs or these may be more widespread. Angiokeratoma corporis diffusum is seen in Fabry disease, a rare X-linked recessive lysosomal storage disorder, as well as other rare inherited metabolic disorders such as fucosidosis, sialidosis, Kanzaki disease, beta mannosidosis, aspartylglycosaminuria, galactosialidosis, and GM1 gangliosidosis.
  • Angiokeratoma of Fordyce – 2-3 mm, smooth, red or violaceous papules are seen on the scrotum or vulva, typically in older individuals. Occasionally these may be larger (up to 5 mm) or warty.
  • Angiokeratoma circumscriptum – One or more plaques of blue-red papules with a verrucous surface develop during childhood, most often on the lower limb. Angiokeratoma circumscriptum has been associated with Klippel-Trenaunay-Weber syndrome (osteohypertrophy of a limb), Cobb syndrome, port-wine stain, cavernous hemangiomas, and arteriovenous fistulas.


D23.9 – Other benign neoplasm of skin, unspecified

254788004 – Angiokeratoma of skin

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Differential Diagnosis & Pitfalls

  • Hemangioma (eg, Microvenular hemangioma)
  • Lobular capillary hemangioma (pyogenic granuloma)
  • Cherry hemangioma
  • Glomangiomas
  • Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu)
  • Blue rubber bleb nevus syndrome
  • Lymphangioma circumscriptum
  • Non-AIDS Kaposi sarcoma
  • Common acquired nevus
  • Spitz nevus
  • Pigmented basal cell carcinoma
  • Melanoma
  • Genital wart

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Last Updated:10/29/2017
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Angiokeratoma in Child
A medical illustration showing key findings of Angiokeratoma (Solitary or Multiple Angiokeratoma)
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