Angiokeratoma of the Scrotum Clinical Presentation

  • Author: Amor Khachemoune, MD, CWS; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Aug 16, 2011
 

History

Patients usually give a history of many years of a 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.[2, 6]
  • Many cases are reported in which help was sought to rule out a sexually transmitted disease or to rule out malignancy.[5, 18, 25]
  • Bleeding from vulval lesions may occur spontaneously, during pregnancy, or after intercourse.[5]
  • Most authors report that lesions are asymptomatic; however, a few describe pain or itching.[29]
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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).[30]
  • 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.[5]
  • The lesions number from 1 to multiple. In a study of 25 women with vulval lesions, 50% of the cases had solitary lesions.[5]
  • Lesions have been reported on the labia majora, shaft of the penis, inner thigh, and lower abdomen. The scrotum is the most common site.[31] See the images below. Image courtesy of Hon Pak, MD, and reviewed by RosImage courtesy of Hon Pak, MD, and reviewed by Ross Levy, MD. Close-up of the eruption. Image courtesy of Hon PaClose-up of the eruption. Image courtesy of Hon Pak, MD, and reviewed by Ross Levy, MD.
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Causes

The role of coexistent venous hypertension, varicocele, or status post radiotherapy remains uncertain and warrants further investigation.

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Contributor Information and Disclosures
Author

Amor Khachemoune, MD, CWS  Mohs Micrographic Surgery, Dermatopathology, 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)

Yoon-Soo Cindy Bae-Harboe, MD  Resident Physician, Department of Dermatology, Boston University School of Medicine, Boston Medical Center

Yoon-Soo Cindy Bae-Harboe, MD is a member of the following medical societies: American Medical Student Association/Foundation

Disclosure: Nothing to disclose.

Marianna Blyumin-Karasik, MD  Resident Physician, Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine

Marianna Blyumin-Karasik, 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.

Meredith M Hancock  Loyola University Chicago Stritch School of Medicine

Meredith M Hancock is a member of the following medical societies: American Medical Women's Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Timothy McCalmont, MD  Director, UCSF Dermatopathology Service, Professor of Clinical Pathology and Dermatology, Departments of Pathology and Dermatology, University of California at San Francisco; Editor-in-Chief, Journal of Cutaneous Pathology

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: Apsara Consulting fee Independent contractor

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside 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.

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.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M 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, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Acknowledgments

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors Joseph J. Shaffer, MBBS, and Vincent A. de Leo, MD, to the development and writing of this article.

References

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

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  23. 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].

  24. Pavone P, Lucenti C, Fraggetta F, Micali G, Incorpora G, Ruggieri M. Congenital lymphedema-lymphangiectasia associated with scrotal angiokeratoma (Fordyce Type) and hearing impairment. J Clin Gastroenterol. Jul 2008;42(6):715-9. [Medline].

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  30. Atherton DJ, and Moss C. Breathnach S, Cox N, et al (Eds). Naevi and other developmental defects, in Burns T : Rook's Textbook of Dermatology. Oxford: Blackwell Science; 2004:pp 15.87-15.90.

  31. Panotte DM. Angiokeratoma: a cause of scrotal bleeding. South Med J. 1985;78:487-488.

  32. Lapins J, Emtestam L, Marcusson JA. Angiokeratomas in Fabry's disease and Fordyce's disease: successful treatment with copper vapour laser. Acta Dermatol Venereol. 1993;73:133-5.

  33. 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].

  34. 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].

  35. Ozdemir M, Baysal I, Engin B, Ozdemir S. Treatment of angiokeratoma of Fordyce with long-pulse neodymium-doped yttrium aluminium garnet laser. Dermatol Surg. Jan 2009;35(1):92-7. [Medline].

  36. Civas E, Koç E, Aksoy B, Aksoy HM. Report of two angiokeratoma of Fordyce cases treated with a 1064 nm long-pulsed Nd:YAG laser. Photodermatol Photoimmunol Photomed. Jun 2009;25(3):166-8. [Medline].

  37. Seo SH, Chin HW, Sung HW. Angiokeratoma of Fordyce treated with 0.5% ethanolamine oleate or 0.25% sodium tetradecyl sulfate. Dermatol Surg. Oct 2010;36(10):1634-7. [Medline].

 
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Image courtesy of Hon Pak, MD, and reviewed by Ross Levy, MD.
Close-up of the eruption. Image courtesy of Hon Pak, MD, and reviewed by Ross Levy, MD.
 
 
 
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