Angiokeratoma of the Scrotum

Updated: Aug 07, 2017
  • Author: Yoon-Soo (Cindy) Bae, MD; Chief Editor: Dirk M Elston, MD  more...
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Overview

Background

In 1896, John Addison Fordyce first described angiokeratomas of Fordyce on the scrotum of a 60-year-old man. [1] Angiokeratomas are typically asymptomatic, 2- to 5-mm, blue-to-red papules with a scaly surface located on the scrotum, shaft of penis, labia majora, inner thigh, or lower abdomen. Histologically, they are composed of ectatic thin-walled vessels in the superficial dermis with overlying epidermal hyperplasia. [2, 3, 4, 5]

"Angiokeratoma" is a broad term that encompassing various asymptomatic hyperkeratotic vascular disorders accompanied by a histologic combination of hyperkeratosis and superficial dermal vascular ectasia. [6] More specifically, angiokeratomas can be categorized into localized and systemic forms.

There are four major localized forms with different presentations. First, solitary papular angiokeratomas typically occurs on the legs. Second, Fordyce-type angiokeratomas are usually localized to the scrotum and vulva. Third, angiokeratoma circumscriptum naviforme is the congenital form that presents as multiple, hyperkeratotic, papular, and plaquelike lesions, usually unilaterally on the lower leg, foot, thigh, buttock, and occasionally elsewhere. Finally, bilateral angiokeratomas, also known as the Mibelli type, occur on the dorsa of the fingers and toes.

The generalized systemic form, angiokeratoma corporis diffusum, is usually associated with metabolic disorders, the most common being Fabry disease or fucosidosis. Although Fabry disease is associated with the generalized presentation, a case report in 2010 recommends considering Fabry disease in all male patients with angiokeratomas, even if localized to the scrotum. [7] Although the pathogenesis and clinical presentation vary, the histologic features are similar for all forms. [2, 8, 9]

Precise epidemiological data are lacking, although estimations have been made. The principal morbidity comes from bleeding, anxiety, and overtreatment due to misdiagnosis by physicians. Usually, these lesions do not require treatment. If treatment is needed, locally destructive methods including electrocoagulation, excision, cryotherapy, or laser therapy may be used. [2, 10]

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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 at an early age. [22]

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Epidemiology

Frequency

The precise incidence of angiokeratomas of Fordyce is unknown, but they are considered common, especially with increasing age. [2, 3, 4]

Race

Most reports on the disease include large case series of angiokeratomas from the United States and Japan, which may at first paint a picture of disease predominantly in whites and in Japanese populations. However, cases in patients of other ethnicities exist but may be underreported.

Sex

There are more reports describing males more often than females, although direct figures of comparison do not exist. Some suggest that incidence in females is just as common as in males, but cases are grossly underreported and underrepresented in the literature. [23]

Age

Cases have been reported ranging from children born with lesions to patients in their sixth decade who develop lesions. [24] One publication on vulval angiokeratomas confirmed by pathology reports showed that 68% of lesions occurred in women aged 20-40 years. [5] A study of 1552 Japanese males found that angiokeratomas occurred at all ages but were most prevalent among people older than 40 years. [15] Prevalence according to this report was as follows [25] :

  • Age 16-20 years - 0.6%
  • Age 21-30 years - 1.5%
  • Age 31-40 years - 6.2%
  • Age 41-50 years - 13.1%
  • Age 51-60 years - 13.4%
  • Age 61-70 years - 15.9%
  • Age 70 years or older - 16.6%
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Prognosis

No fatalities have been reported from this condition. The most significant morbidity comes from bleeding. [5] The papules can bleed spontaneously if traumatized or during intercourse. Many of the reports describe patient concern that the lesions represent a sexually transmitted disease. [26]

Spontaneous resolution of angiokeratomas has not been observed in the literature; these lesions persist unless treated. Patients with multiple angiokeratomas are more likely to have recurrences after treatment than those with few or solitary angiokeratomas.

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Patient Education

In most cases of angiokeratoma, the patient, and when appropriate the partner, should be reassured that the condition is common, benign, and does not represent any form of sexually transmitted disease. More lesions may develop with increasing age.

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