eMedicine Specialties > Dermatology > Benign Neoplasms

Angiokeratoma of the Scrotum

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
Coauthor(s): Marianna Larisa Blyumin, MD, Staff Physician, Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine
Contributor Information and Disclosures

Updated: Jan 15, 2008

Introduction

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%

Clinical

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.

More on Angiokeratoma of the Scrotum

Overview: Angiokeratoma of the Scrotum
Differential Diagnoses & Workup: Angiokeratoma of the Scrotum
Treatment & Medication: Angiokeratoma of the Scrotum
Follow-up: Angiokeratoma of the Scrotum
Multimedia: Angiokeratoma of the Scrotum
References

References

  1. Erkek E, Basar MM, Bagci Y, Karaduman A, Bilen CY, Gokoz A. Fordyce angiokeratomas as clues to local venous hypertension. Arch Dermatol. Oct 2005;141(10):1325-6. [Medline].

  2. Agger P, Osmundsen PE. Angiokeratoma of the scrotum (Fordyce). A case report on response to surgical treatment of varicocele. Acta Derm Venereol. 1970;50(3):221-4. [Medline].

  3. Orvieto R, Alcalay J, Leibovitz I, Nehama H. Lack of association between varicocele and angiokeratoma of the scrotum (Fordyce). Mil Med. Jul 1994;159(7):523-4. [Medline].

  4. Leis-Dosil VM, Alijo-Serrano F, Aviles-Izquierdo JA, Lazaro-Ochaita P, Lecona-Echeverria M. Angiokeratoma of the glans penis: clinical, histopathological and dermoscopic correlation. Dermatol Online J. 2007;13(2):19. [Medline].

  5. Al-Mutairi N, Joshi A, Nour-Eldin O. Naevus lipomatosus cutaneous superficialis of Hoffmann-Zurhelle with angiokeratoma of Fordyce. Acta Derm Venereol. 2006;86(1):92-3. [Medline].

  6. Jansen T, Bechara FG, Stücker M, Altmeyer P. Angiokeratoma of the scrotum (Fordyce type) associated with angiokeratoma of the oral cavity. Acta Derm Venereol. 2002;82(3):208-10. [Medline].

  7. Caputo R, Passoni E, Cavicchini S. Papular xanthoma associated with angiokeratoma of Fordyce: considerations on the nosography of this rare non-Langerhans cell histiocytoxanthomatosis. Dermatology. 2003;206(2):165-8. [Medline].

  8. Lapins J, Emtestam L, Marcusson JA. Angiokeratomas in Fabry's disease and Fordyce's disease: successful treatment with copper vapour laser. Acta Derm Venereol. Apr 1993;73(2):133-5. [Medline].

  9. Occella C, Bleidl D, Rampini P, Schiazza L, Rampini E. Argon laser treatment of cutaneous multiple angiokeratomas. Dermatol Surg. Feb 1995;21(2):170-2. [Medline].

  10. Bechara FG, Jansen T, Wilmert M, Altmeyer P, Hoffmann K. Angiokeratoma Fordyce of the glans penis: combined treatment with erbium: YAG and 532 nm KTP (frequency doubled neodynium: YAG) laser. J Dermatol. Nov 2004;31(11):943-5. [Medline].

  11. Lapidoth M, Ad-El D, David M, Azaria R. Treatment of angiokeratoma of Fordyce with pulsed dye laser. Dermatol Surg. Sep 2006;32(9):1147-50. [Medline].

  12. Blair C. Angiokeratoma of the vulva. Br J Dermatol. Sep 1970;83(3):409-11. [Medline].

  13. Carrasco L, Izquierdo MJ, Farina MC, et al. Strawberry glans penis: a rare manifestation of angiokeratomas involving the glans penis. Br J Dermatol. Jun 2000;142(6):1256-7. [Medline].

  14. Fordyce J. Angiokeratoma of the scrotum. J Cutan Dis. 1986;14:81.

  15. Hisa T, Taniguchi S, Goto Y, Teramae H, Osato K, Kakudo K, et al. Scrotal angiokeratoma in a young man. Acta Derm Venereol. May 1996;76(3):248-9. [Medline].

  16. Imperial R, Helwig EB. Angiokeratoma of the scrotum (Fordyce type). J Urol. Sep 1967;98(3):379-87. [Medline].

  17. Imperial R, Helwig EB. Angiokeratoma of the vulva. Obstet Gynecol. Mar 1967;29(3):307-12. [Medline].

  18. Izaki M. Angiokeratoma of the Scrotum (Fordyce). Keio J Med. 1952;1:61-8.

  19. Karthikeyan K, Sethuraman G, Thappa DM. Angiokeratoma of the oral cavity and scrotum. J Dermatol. Feb 2000;27(2):131-2. [Medline].

  20. Patrizi A, Neri I, Trevisi P, Landi C, Bardazzi F. Congenital angiokeratoma of Fordyce. J Eur Acad Dermatol Venereol. Mar 1998;10(2):195-6. [Medline].

  21. Schiller PI, Itin PH. Angiokeratomas: an update. Dermatology. 1996;193(4):275-82. [Medline].

Further Reading

Keywords

angiokeratoma of Fordyce, Fordyce angiokeratoma, vulvar angiokeratoma

Contributor Information and Disclosures

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, MD, CWS 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
Disclosure: Nothing to disclose.

Coauthor(s)

Marianna Larisa Blyumin, MD, Staff Physician, Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine
Marianna Larisa Blyumin, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Medical Women's Association, American Society for Dermatologic Surgery, Dermatology Foundation, Medical Dermatology Society, and Women's Dermatologic Society
Disclosure: Nothing to disclose.

Medical Editor

Timothy McCalmont, MD, Director, UCSF Dermatopathology Service, Professor of Clinical Pathology and Dermatology, Departments of Pathology and Dermatology, University of California at San Francisco
Timothy McCalmont, MD is a member of the following medical societies: Alpha Omega Alpha, American Medical Association, American Society of Dermatopathology, California Medical Association, College of American Pathologists, and United States and Canadian Academy of Pathology
Disclosure: Nothing to disclose.

Pharmacy Editor

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
David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Managing Editor

Edward F Chan, MD, Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine
Edward F Chan, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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