A 26-year-old man presented to the emergency department after a spontaneous 30 min bleed from his scrotal skin. He showed no other symptoms and denied any past medical history. He was exclusively sexually active, systemically well and haemodynamically stable. There were numerous (>50) 1-2 mm dark red, erythematous papules over the scrotum, sparing the shaft of penis, inner thigh and abdomen. A small area of blood marked the bleeding spot as a single papule. A diagnosis of angiokeratoma of the scrotum (Fordyce) was made and potential precipitants such as intra-abdominal masses, urinary tract tumours, varicoceles, hernias and angiokeratoma corporis diffusum (Fabry syndrome) were excluded. He was discharged with dermatology follow-up with a view to local laser treatment. The important differential diagnoses are angiokeratoma corporis diffusum and malignant melanoma (nodular type). In females, Fordyce angiokeratoma are distributed on labia majora.

Angiokeratoma circumscriptum arranged in a systematized band-like pattern suggesting mosaicism. J Dermatol. 2006 Jul;33(7):489-91.

A 10-year-old girl with vascular lesions that had been present since birth is reported. Initially, small red macules were limited to the legs, but later the lesions became more extensive. A hyperkeratotic aspect of the lesional skin surface had developed at the age of 2 years. On clinical examination, hyperkeratotic vascular lesions in a band-like distribution on the trunk, legs and face were observed. The type of lesion varied from discrete macules with no or slight hyperkeratosis to confluent, protruding verrucous plaques. The clinical and histopathological findings were consistent with a diagnosis of angiokeratoma circumscriptum. The systematized band-like arrangement observed in the present case strongly supports the concept that angiokeratoma circumscriptum reflects a mosaic state of a mutation that is so far unknown.

Angiokeratoma of tongue: a series of 14 cases.J Pak Med Assoc. 2006 Jun;56(6):285-7.

Angiokeratomas (AC) are vascular lesions which are defined histologically as one or more dilated blood vessels lying directly subepidermal and showing an epidermal proliferative reaction with ectatic capillaries in the papillary dermis. Only three other cases of isolated mucosal angiokeratoma have been reported in the indexed literature. We reviewed all cases of angiokeratoma located on the tongue, diagnosed in our department during a study period of 10 years (1995-2005). Histologically all 14 cases showed dilated and congested blood vessels in the upper papillary dermis. They lack deep dermal involvement. Hyperkeratosis and acanthosis were also seen in most of the cases. No clinical data was available to assess systemic disease. A higher incidence of of AC in tongue is seen in our study.