Angiokeratoma (overview) D23.L

• Heterogeneous group of congenital (naevi) or acquired hemangiomas or vascular ectasias in which the hyperkeratotic, wart-like surface was originally decisive for the naming. However, hyperkeratosis or the wart-like clinical aspect is often not a leading feature or is missing completely. Therefore, a uniform etiological bracket is missing, which defines the generic term "angiokeratome" for this heterogeneous group of agiomas.
• Most so-called angiokeratomas are histologically capillary or cavernous hemangiomas or mixed images or, as in the case of scrotal or vulval angiokeratomas ( Angiokeratoma scroti et vulvae), phlebectasia.
• The aetiological classification of Mibelli's angiokeratoma, which occurs mainly in adolescents on fingers and toes, is not clear.
• Angiokeratoma corporis diffusum as well as fucosidosis and beta-mannosidosis are dermatological symptoms of lysosomal storage diseases (lack 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.