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Dermatology > BENIGN NEOPLASMS
Angiokeratoma of the Scrotum
Article Last Updated: Jan 15, 2008
AUTHOR AND EDITOR INFORMATION
Section 1 of 12  
Author: Amor Khachemoune, MD, CWS, Clinical Instructor, Mohs Micrographic Surgery, Department of Dermatology, State University of New York Downstate Medical Center; Consulting Staff, Department of Dermatology, Veterans Affairs Medical Center of Brooklyn
Amor Khachemoune is a member of the following medical societies: American Academy of Dermatology, American Academy of Wound Management, American College of Mohs Micrographic Surgery and Cutaneous Oncology, American Medical Association, American Society for Dermatologic Surgery, and American Society for Laser Medicine and Surgery
Coauthor(s):
Marianna Larisa Blyumin, MD, Staff Physician, Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine
Editors: Timothy McCalmont, MD, Director, UCSF Dermatopathology Service, Professor of Clinical Pathology and Dermatology, Departments of Pathology and Dermatology, University of California at San Francisco; David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Director, Division of Dermatology, Scott and White Clinic; Director Dermatology Residency Training Program, Scott and White Clinic; Edward F Chan, MD, Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University; Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Author and Editor Disclosure
Synonyms and related keywords:
angiokeratoma of Fordyce, Fordyce angiokeratoma, vulvar angiokeratoma
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 ectatic thin-walled vessels 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, cryotherapy, or laser therapy 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.1 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). 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,2 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.3 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.
Penile and vulvar angiokeratomas have also been noted status post radiation treatment of genitourinary malignancy.4
Angiokeratomas of Fordyce have also been reported in association with nevus lipomatosus,5 oral mucosal angiokeratomas,6 and papular xanthoma.7
Frequency
International
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 whites and in Japanese populations. Cases in blacks exist but are few in number. The only publications on vulval lesions have been in white 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. The only publication on vulval lesions, identified by pathology reports of removed lesions, showed that 68% of lesions occurred in women aged 20-40 years. 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:
- 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%
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 or to rule out malignancy.
- 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.
Physical
- 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 venous hypertension, varicocele, and status post radiotherapy remain uncertain and warrants further investigation.
Angiokeratoma Corporis Diffusum (Fabry Syndrome)
Cherry Hemangioma
Malignant Melanoma
Nevi, Melanocytic
Warts, Genital
Other Problems to Be Considered
Patients with history of genitourinary malignancy and subsequent surgical or radiation therapy may develop angiokeratomas of Fordyce. These lesions may be alarming as potential recurrence or metastatic presentation of the cancer. Performing a biopsy and reassuring patients of the benign nature of these lesions is important.
Imaging Studies
- Imaging studies are not warranted in the evaluation of this condition.
Procedures
- Dermoscopy can assist with the diagnosis. Angiokeratoma is characterized by large, well-demarcated, round-to-oval, and red-to-black areas, which are lacunar. In addition, a white surrounding veil corresponds to the acanthotic and hyperkeratotic epidermis.4
- If the diagnosis is in doubt, then a skin biopsy may be needed.
Histologic Findings
Numerous dilated, thin-walled vessels are positioned in the papillary dermis or superficial submucosa, with an intimate relationship to the overlying acanthotic epidermis with overlying parakeratosis. In addition to elongation of the rete ridges, the epithelium is usually hyperkeratotic. Thrombosis of the vascular spaces is common, and frequently there is recanalization of occluded vascular spaces, creating the pathologic pattern known as papillary endothelial hyperplasia (Masson lesion).
Medical Care
The importance of these lesions was well summarized by Bean, "These varicules should be known so that we can allay the fears of old men, many of whom have worries enough already." If the lesions are an incidental finding or are asymptomatic, then the patient can be reassured about the lesions benign nature. If there is concern over bleeding or cosmetic appearance, then several surgical treatment options exist.
Surgical Care
- Excision: This is not practical if more than a few lesions exist. However, excision can be performed under local anesthesia with a good cosmetic result.
- Cryotherapy: Application of liquid nitrogen has been used with resolution of the lesions but with residual hypopigmentation and scarring.
- Electrocautery: Light electrocoagulation has been used with or without local anesthesia to produce effective resolution of lesions.
- Laser: Successful resolution has been reported with single treatments using both the 578-nm copper laser8 and the argon laser,9 resulting in minimal scarring. A 2004 study showed benefit using a 532-nm potassium-titanyl-phosphate (KTP) laser.10 Another study in 2006 evaluated the efficacy of pulsed-dye laser in 12 patients with scrotal angiokeratomas.11 The results demonstrated good-to-excellent response in all patients with transient purpura and minimal procedural bleeding as the only adverse effects.
Consultations
- Consult a dermatologist if the diagnosis is in doubt; alternatively, a biopsy can be performed on the lesions and can be submitted to a dermatopathology laboratory for microscopic diagnosis.
- Consult a urologist if suspicion of a varicocele is present.
Angiokeratomas of Fordyce are a benign neoplasms and are not amenable to drug therapy.
Further Outpatient Care
- If a surgical procedure is performed, follow-up care at 3 months post treatment is indicated to assess the cosmetic result and to look for recurrences.
Complications
- As mentioned previously, bleeding is the only major complication that these patients experience.
Prognosis
- Spontaneous resolution is not described. The lesions will persist unless treated. Patients with multiple lesions are more likely to have recurrences after treatment than those with few or solitary lesions.
Patient Education
- In most cases, 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.
Medical/Legal Pitfalls
- 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 (dermoscopy 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.
The authors and editors of eMedicine gratefully acknowledge the contributions of previous authors Joseph J. Shaffer, MBBS, Vincent A. de Leo, MD, to the development and writing of this article.
| Media file 1:
Image courtesy of Hon Pak, MD, and reviewed by Ross Levy, MD. |
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| Media file 2:
Close-up of the eruption in patient in Image 1. Image courtesy of Hon Pak, MD, and reviewed by Ross Levy, MD. |
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Angiokeratoma of the Scrotum excerpt Article Last Updated: Jan 15, 2008
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