Join Date: Nov 2002
here is even more information:
Background: In 1896, John Addison Fordyce first described angiokeratomas of Fordyce on the scrotum of a 60-year-old man. 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 dilated ectatic capillaries in the superficial dermis with overlying epidermal hyperplasia.
Precise data on their frequency and distribution are lacking, although estimations have been made. The principle morbidity comes from bleeding, anxiety, and overtreatment due to misdiagnosis by physicians. Usually, they do not require treatment. If treatment is needed, then locally destructive methods including laser, electrocoagulation, excision, or cryotherapy may be used.
Pathophysiology: The pathophysiology of angiokeratomas remains unknown, although it has been proposed that an increased venous pressure may contribute to their formation.
There are many reports of angiokeratomas occurring in the presence of a varicocele or other conditions of increased venous pressure (eg, hernias, epididymal tumors, urinary system tumors). One series reports that up to two thirds of patients have associated conditions. One case exists where the varicocele was treated and the angiokeratomas resolved, and one report exists in which varicocele treatment failed to produce improvement.
Equally, there are many cases where no cause for increased venous pressure was found. In a study of 435 military recruits aged 18-19 years, 10% (n=46) were found to have varicoceles; none had angiokeratomas. They also surveyed 30 soldiers aged 45-55 years with varicoceles but found no angiokeratomas. They propose that the coexistence of varicocele and angiokeratomas are coincidental. Similarly, a study of 1552 Japanese males found no history of any venous obstructive disorders.
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.
* Internationally: The precise incidence of angiokeratomas of Fordyce is unknown, but they are considered common especially with increasing age.
Mortality/Morbidity: No fatalities have been reported from this condition. The most significant morbidity comes from bleeding. The papules can bleed spontaneously if traumatized or during intercourse. Many of the cases report patients concern that the lesions represent a sexually transmitted disease.
Race: Large series of angiokeratomas have been reported from America and Japan, which give a picture of disease predominantly in Caucasians and in Japanese populations. Cases in blacks exist but are few in number. The only publications on vulval lesions have been in Caucasian women.
Sex: Males have been reported far more often than females, although direct figures of comparison do not exist. It has been commented that female angiokeratomas are probably as common as males but grossly underreported and underrepresented in the literature.
Age: Cases have been reported ranging from children born with lesions to lesions developing in patients in their sixth decade. A study of 1552 Japanese males found that the condition occurred at all ages but was most prevalent among people older than 40 years. Prevalence was as follows:
History: Patients usually give a history of many years of progressive appearance of asymptomatic papules on the scrotum.
* The patient may not be aware of the lesions, and bleeding (spontaneous, after intercourse or scratching) may be the first presentation causing the patient to seek medical help.
* Many cases are reported where help was sought to rule out a sexually transmitted disease.
* Bleeding from vulval lesions may occur spontaneously, during pregnancy, or after intercourse.
* Most authors report that lesions are asymptomatic; however, a few describe pain or itching.
* Fordyce angiokeratomas appear as black, blue, or dark red, dome-shaped papules ranging from 1-6 mm in diameter, with a mean of 3 mm. The overlying surface may show slight scales (hyperkeratosis).
* Reports suggest that in younger patients the lesions tend to be smaller, more erythematous, and less hyperkeratotic. Older patients have larger, darker lesions (blue/black) with overlying scales.
* The lesions number from 1 to many (>100). In a study of 25 women with vulval lesions, 50% of the cases had solitary lesions.
* Lesions have been reported on the labia majora, shaft of the penis, inner thigh, and lower abdomen. The scrotum is the most common site.
Causes: The role of coexistent varicocele remains uncertain and warrants further investigation.
* The most ominous clinical differential diagnostic consideration is malignant melanoma. Angiokeratomas are composed of superficial vessels immediately subjacent to the epidermis, and because of the common occurrence of intraepidermal hemorrhage and subepidermal thrombosis, the lesions appear deeply pigmented or black from a clinical standpoint, and thus simulate the clinical appearance of melanoma. If the diagnosis is in doubt, then the patient should be referred to a dermatologist to examine the lesion and to perform a biopsy, if needed. Epiluminescent examination (episcope examination) also can be useful in the distinction of a vascular from a melanocytic neoplasm.
* Fordyce angiokeratomas also must be distinguished from angiokeratomas of Fabry disease. Patients with Fabry disease may report lancinating limb pain or a history of renal disease. Routine histology sometimes demonstrates vacuoles within endothelial cells in patients with Fabry disease. Electron microscopy may demonstrate lamellated inclusion bodies within endothelial cells. Fabry disease should be considered when angiokeratomas are present on the shaft, sacrum, or suprapubic areas in addition to the scrotum.