English: Pathophysiology

The pathophysiology of angiokeratomas remains unknown, although some authors believe increased venous pressure may contribute to their formation. [11] Many reports describe angiokeratomas occurring in the presence of a varicocele or other conditions of increased venous pressure (eg, hernias, epididymal tumors, urinary system tumors, trauma, and thrombophlebitis). [10] One series reports that up to half of patients with angiokeratomas have associated venous pressure conditions. [12] One report describes treatment of the varicocele followed by resolution of the angiokeratomas, [10] although another report describes varicocele treatment followed by no improvement in the angiokeratomas. [13] Other proposed causative factors include acute or chronic trauma and nevoid or vascular malformations. [14] However, some authors contend that the coexistence of varicoceles and angiokeratomas is coincidental, as many cases have been described in which no cause for increased venous pressure was found. [13] In a study of 435 military recruits aged 18-19 years, 10% (n = 46) were found to have varicoceles, but no angiokeratomas. They also surveyed 30 soldiers aged 45-55 years with varicoceles but again found no angiokeratomas. [13] Similarly, a study of 1552 Japanese males found no history of any venous obstructive disorders. [15] The literature notes several associated predisposing factors with this disease. In a study of vulval angiokeratomas, 54% of patients were noted to have a predisposing factor (eg, pregnancy, vulval varicosity, post partum, post hysterectomy), while the rest had none. [12] There is also a report of this condition occurring in conjunction with chronic infection with human papillomavirus in a 25-year-old woman. [16] In addition, there may be iatrogenic predisposing factors as well. Several reports detail that radiation therapy for treatment of genitourinary malignancy may be associated with the formation of angiokeratomas of the penis and the vulva. [17] Another case report describes a man with a recurrent penile angiokeratoma after surgery. [18] Angiokeratomas of Fordyce have also been reported in association with nevus lipomatosus, [19] oral mucosal angiokeratomas, [8, 20] and papular xanthoma. [21]

Interestingly, a case of a 16-year-old boy with congenital lymphangiectasia-lymphedema born to consanguineous parents was found to have angiokeratoma of the scrotum and the penis