Angiokeratoma (overview) D23.L

• Heterogeneous group of congenital (vascular hamartomas) or acquired hemangiomas or vascular ectasias, in which the hyperkeratotic, warty surface was originally decisive for the naming. However, the hyperkeratosis or the warty clinical aspect are often not a leading feature or are completely absent. Thus, a uniform etiologic bracket defining the generic term "angiokeratoma" for this heterogeneous group of agiomas is also lacking.
• Most of the so-called angiokeratomas are histologically capillary or cavernous hemangiomas or mixed patterns or, as in the case of scrotal or vulvar angiokeratomas ( angiokeratoma scroti et vulvae), phlebectasias.
• The etiological assignment of angiokeratoma mibelli is unclear. It is said to occur mainly in adolescents on fingers and toes.
• Angiokeratoma corporis diffusum as well as fucosidosis and beta-mannosidosis are dermatological symptoms of lysosomal storage diseases (deficiency of alpha- or beta-galactosidase; fucosidase, mannosidase).

Angiokeratomas include:

• Angiokeratoma Mibelli
• Angiokeratoma scroti et vulvae (Fordyce)
• Angiokeratome, solitary
• angiokeratoma circumscriptum
• Angioeratomas, acral pseudolymphomatous childhood (entity disputed).

Angiokeratomas are observed in the following lysosomal storage diseases and shape the clinical picture dermatologically:

• Angiokeratoma corporis diffusum (Fabry disease - alpha galactosidase deficiency)
• Fucosidosis
• Beta-mannosidosis
• Aspartyl glucosaminuria.