The New England Journal of Medicine: Angiokeratoma Corporis Diffusum Universale (Fabry's Disease) in Two Brothers ANGIOKERATOMA corporis diffusum universale, or Fabry's disease, was regarded as a dcrmatologie curiosity until a description by Ruiter and Pompen 1 from the Netherlands in 1939 suggested that it was a ... Angiokeratomas are benign, red-blue wart-like skin lesions that are more common in older individuals. They are classified according to body site, and some types have a male or female predominance.

Understanding the Context

Introduction Angiokeratoma presents as a blood-filled papule which may bleed following trauma. There are a range of clinical presentations ranging from isolated lesions of little consequence to widespread lesions associated with Fabry disease. Histology of angiokeratoma In angiokeratoma, the histopathology shows a vascular lesion in the superficial dermis which extends into the epidermis ... View pictures of angiokeratoma in the gallery below.

Key Insights

An angiokeratoma is a benign capillary ectasia in the superficial dermis that presents as an asymptomatic blue-red hyperkeratotic papule anywhere on the skin. Fabry disease is a rare inherited lysosomal storage disorder [1]. It is also known as Anderson–Fabry disease and angiokeratoma corporis diffusum. Fabry disease causes clusters of angiokeratomas (small, dark red spots on the skin) and many systemic symptoms due to the deposition of globotriaosylceramide (Gb3) in multiple organs. Angiokeratoma presents as a blood-filled papule which may bleed following trauma.

Final Thoughts

There are a range of clinical presentations ranging from isolated lesions of little consequence to widespread lesions associated with Fabry disease. Created 2008. Learning objectives Describe clinical features and management of dermal and subcutaneous tumours of vascular, neural, fibrous, metastatic, or other origin. Vascular tumours Angiokeratoma Angiokeratoma is a scaly vascular papule due to epidermal proliferation encircling dilated vessels. Angiokeratoma may be solitary or diffuse. Multiple lesions may be genital (Fordyce), acral ...