ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyReferencesView all Images (38)
Angiokeratoma in Adult
Print
Other Resources UpToDate PubMed

Angiokeratoma in Adult

Print Images (38)
Contributors: Tracy Lu, Sarah Hocker DO, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

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.

Codes

ICD10CM:
D23.9 – Other benign neoplasm of skin, unspecified

SNOMEDCT:
254788004 – Angiokeratoma of skin

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

References

Subscription Required

Last Updated: 10/30/2017
Copyright © 2018 VisualDx®. All rights reserved.
Angiokeratoma in Adult
Print 38 Images
View all Images (38)
(with subscription)
Angiokeratoma (Solitary or Multiple Angiokeratoma)
Copyright © 2018 VisualDx®. All rights reserved.