Angiokeratomas (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 vulvar angiokeratomas ( Angiokeratoma scroti et vulvae), vascular ectasia.

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).

The term "angiokeratome" covers the following clinical pictures:

• Angiokeratoma Mibelli
• Angiokeratoma scroti et vulvae (Fordyce)
• Angiokeratome, solitary
• Multiple (non-syndromal) angiokeratomas (Calzavara-Pinton)
• 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.