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, 10]}

Many cases are reported in which help was sought to rule out a sexually transmitted disease or to rule out malignancy.^{[5, 30, 29]}

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.^[31]

Physical Examination

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).^[32]

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 can present as singular or 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, corona of the glans penis, inner thigh, and lower abdomen. The scrotum is the most common site.^{[33, 34]}

See the images below.

Image courtesy of Hon Pak, MD, and reviewed by Ross Levy, MD.

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

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Differential Diagnoses

Fordyce JA. Angiokeratoma of the scrotum. J Cutan Genitourin Dis. 1896. 14:81-7.
Schiller PI, Itin PH. Angiokeratomas: an update. Dermatology. 1996. 193(4):275-82. [QxMD MEDLINE Link].
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. 2000 Jun. 142(6):1256-7. [QxMD MEDLINE Link].
Gioglio L, Porta C, Moroni M, Nastasi G, Gangarossa I. Scrotal angiokeratoma (Fordyce): histopathological and ultrastructural findings. Histol Histopathol. 1992 Jan. 7(1):47-55. [QxMD MEDLINE Link].
Imperial R, Helwig EB. Angiokeratoma of the vulva. Obstet Gynecol. 1967 Mar. 29(3):307-12. [QxMD MEDLINE Link].
Yamazaki M, Hiruma M, Irie H, Ishibashi A. Angiokeratoma of the clitoris: a subtype of angiokeratoma vulvae. J Dermatol. 1992 Sep. 19(9):553-5. [QxMD MEDLINE Link].
Hogarth V, Dhoat S, Mehta AB, Orteu CH. Late-onset Fabry disease associated with angiokeratoma of Fordyce and multiple cherry angiomas. Clin Exp Dermatol. 2011 Jul. 36(5):506-8. [QxMD MEDLINE Link].
Jansen T, Bechara FG, Stucker M, Altmeyer P. Angiokeratoma of the scrotum (Fordyce type) associated with angiokeratoma of the oral cavity. Acta Derm Venereol. 2002. 82(3):208-10. [QxMD MEDLINE Link].
Muller C, James WD. Angiokeratoma of Fordyce as a cause of red scrotum. Cutis. 2002. 69:50-51.
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. [QxMD MEDLINE Link].
González-López MA, Consuegra G, Lacalle M, et al. Unilateral angiokeratoma of the scrotum (Fordyce's type) associated with a contralateral varicocele. Indian J Dermatol Venereol Leprol. 2017 Jul-Aug. 83 (4):470-472. [QxMD MEDLINE Link]. [Full Text].
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 May 1. 13(2):19. [QxMD MEDLINE Link].
Pianezza ML, Singh D, Van der Kwast T, Jarvi K. Rare case of recurrent angiokeratoma of Fordyce on penile shaft. Urology. 2006/10. 68(4):891.e1-3.
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. 2004 Nov. 31(11):943-5. [QxMD MEDLINE Link].
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.
Occella C, Bleidl D, Rampini P, Schiazza L, Rampini E. Argon laser treatment of cutaneous multiple angiokeratomas. Dermatol Surg. 1995 Feb. 21(2):170-2. [QxMD MEDLINE Link].
Erkek E, Basar MM, Bagci Y, Karaduman A, Bilen CY, Gokoz A. Fordyce angiokeratomas as clues to local venous hypertension. Arch Dermatol. 2005 Oct. 141(10):1325-6. [QxMD MEDLINE Link].
Imperial R, Helwig EB. Angiokeratoma of the scrotum (Fordyce type). J Urol. 1967 Sep. 98(3):379-87. [QxMD MEDLINE Link].
Orvieto R, Alcalay J, Leibovitz I, Nehama H. Lack of association between varicocele and angiokeratoma of the scrotum (Fordyce). Mil Med. 1994 Jul. 159(7):523-4. [QxMD MEDLINE Link].
McNeely TB. Angiokeratoma of the clitoris. Arch Pathol Lab Med. 1992 Aug. 116(8):880-1. [QxMD MEDLINE Link].
Izaki M. Angiokeratoma of the Scrotum (Fordyce). Keio J Med. 1952. 1:61-8.
Baruah J, Roy KK, Rahman SM, Kumar S, Pushparaj M, Mirdha AR. Angiokeratoma of vulva with coexisting human papilloma virus infection: a case report. Arch Gynecol Obstet. 2008 Aug. 278(2):165-7. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Karthikeyan K, Sethuraman G, Thappa DM. Angiokeratoma of the oral cavity and scrotum. J Dermatol. 2000 Feb. 27(2):131-2. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
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. 2008 Jul. 42(6):715-9. [QxMD MEDLINE Link].
Blair C. Angiokeratoma of the vulva. Br J Dermatol. 1970 Sep. 83(3):409-11. [QxMD MEDLINE Link].
Patrizi A, Neri I, Trevisi P, Landi C, Bardazzi F. Congenital angiokeratoma of Fordyce. J Eur Acad Dermatol Venereol. 1998 Mar. 10(2):195-6. [QxMD MEDLINE Link].
Hisa T, Taniguchi S, Goto Y, et al. Scrotal angiokeratoma in a young man. Acta Derm Venereol. 1996 May. 76(3):248-9. [QxMD MEDLINE Link].
Malalasekera AP, Goddard JC, Terry TR. Angiokeratoma of Fordyce simulating recurrent penile cancer. Urology. 2007 Mar. 69(3):576.e13-4. [QxMD MEDLINE Link].
Taniguchi S, Inoue A, Hamada T. Angiokeratoma of Fordyce: a cause of scrotal bleeding. Br J Urol. 1994 May. 73(5):589-90. [QxMD MEDLINE Link].
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.
Panotte DM. Angiokeratoma: a cause of scrotal bleeding. South Med J. 1985. 78:487-488.
Feramisco JD, Fournier JB, Zedek DC, Venna SS. Eruptive angiokeratomas on the glans penis. Dermatol Online J. 2009 Oct 15. 15(10):14. [QxMD MEDLINE Link].
Ghosh SK, Ghosh S, Agarwal M. Multiple giant angiokeratoma of Fordyce on the shaft of the penis masquerading as keratoacanthoma. An Bras Dermatol. 2015 May-Jun. 90 (3 Suppl 1):150-2. [QxMD MEDLINE Link].
Lapidoth M, Ad-El D, David M, Azaria R. Treatment of angiokeratoma of Fordyce with pulsed dye laser. Dermatol Surg. 2006 Sep. 32(9):1147-50. [QxMD MEDLINE Link].
Cohen PR. A Case Report of Scrotal Rejuvenation: Laser Treatment of Angiokeratomas of the Scrotum. Dermatol Ther (Heidelb). 2019 Mar. 9 (1):185-192. [QxMD MEDLINE Link]. [Full Text].
Ozdemir M, Baysal I, Engin B, Ozdemir S. Treatment of angiokeratoma of Fordyce with long-pulse neodymium-doped yttrium aluminium garnet laser. Dermatol Surg. 2009 Jan. 35(1):92-7. [QxMD MEDLINE Link].
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. 2009 Jun. 25(3):166-8. [QxMD MEDLINE Link].
Baumgartner J, Šimaljaková M Mha. Genital angiokeratomas of Fordyce 595-nm variable-pulse pulsed dye laser treatment. J Cosmet Laser Ther. 2017 Jun 30. [QxMD MEDLINE Link].
Seo SH, Chin HW, Sung HW. Angiokeratoma of Fordyce treated with 0.5% ethanolamine oleate or 0.25% sodium tetradecyl sulfate. Dermatol Surg. 2010 Oct. 36(10):1634-7. [QxMD MEDLINE Link].
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. 2004 Nov. 31(11):943-5. [QxMD MEDLINE Link].
Yang CH, Ohara K. Successful surgical treatment of verrucous hemangioma: a combined approach. Dermatol Surg. 2002 Oct. 28(10):913-19; discussion 920. [QxMD MEDLINE Link].
Yigiter M, Arda IS, Tosun E, Celik M, Hiçsönmez A. Angiokeratoma of clitoris: a rare lesion in an adolescent girl. Urology. 2008 Apr. 71(4):604-6. [QxMD MEDLINE Link].

Media Gallery

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.

of 2

Author

Yoon-Soo (Cindy) Bae, MD Clinical Assistant Professor, Ronald O Perelman Department of Dermatology, New York University School of Medicine; Procedural Dermatologist, Laser and Skin Surgery Center of New York

Yoon-Soo (Cindy) Bae, MD is a member of the following medical societies: American Academy of Dermatology, American College of Mohs Surgery, American Medical Association, American Society for Dermatologic Surgery, American Society for Laser Medicine and Surgery, Massachusetts Medical Society, Women's Dermatologic Society, New England Dermatological Society

Disclosure: Nothing to disclose.

Coauthor(s)

Marianna Blyumin-Karasik, MD Dermatologist, Minars Dermatology; Consulting Dermatologist, Memorial Regional Hospital; Voluntary Instructor in Dermatology, Department of Dermatology and Cutaneous Surgery, University of Miami, Leonard M Miller School of Medicine

Marianna Blyumin-Karasik, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, Women's Dermatologic Society, Dermatology Foundation

Disclosure: Nothing to disclose.

Meredith M Hancock, MD Resident Physician, Department of Dermatology, Marshfield Clinic/Saint Joseph’s Hospital

Meredith M Hancock, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for Laser Medicine and Surgery, Society for Pediatric Dermatology, Women's Dermatologic Society, National Psoriasis Foundation, Medical Dermatology Society

Disclosure: Nothing to disclose.

Edward Bae, MD Resident Physician, Department of Dermatology, Roger Williams Medical Center

Edward Bae, MD is a member of the following medical societies: Gold Humanism Honor Society

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Former Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Military Dermatologists, Phi Beta Kappa, Texas Dermatological Society

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, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

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

Disclosure: Nothing to disclose.

Additional Contributors

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, United States and Canadian Academy of Pathology

Disclosure: Received consulting fee from Apsara for independent contractor.

Acknowledgements

Vincent A De Leo, MD Clinical Professor of Dermatology, Department of Dermatology, College of Physicians and Surgeons of Columbia University; Chairman, Department of Dermatology, Director of Dermatology Residency Training Program, St Luke's-Roosevelt Hospital Center; Chairman, Department of Dermatology, Beth Israel Medical Center

Vincent A De Leo, MD is a member of the following medical societies: American Academy of Dermatology, American College of Occupational and Environmental Medicine, American Contact Dermatitis Society, American Dermatological Association, American Medical Association, American Society for Photobiology, Dermatology Foundation, New York Academy of Medicine, New York County Medical Society, Photomedicine Society, Society for Investigative Dermatology,Society of Toxicology, and Women's Dermatologic Society

Disclosure: estee lauder Consulting fee Consulting; laroche posay Consulting fee Consulting; schering plough Consulting fee Consulting; pfizer Consulting fee Consulting; orfagen Grant/research funds study – clinical

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.

Joseph J Shaffer, MBBS Fellow, Dermatologic Surgery, Department of Cutaneous Surgery, Fairview University Medical Center

Joseph J Shaffer is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.