|LETTER TO EDITOR
|Year : 2014 | Volume
| Issue : 1 | Page : 46-48
Multiple angiokeratomas of Fordyce: Lesions at an unusual site causing a diagnostic dilemma
Sharmila Dudani1, Rajendra Maskara2
1 Department of Pathology, Army College of Medical Sciences, Delhi Cantonment, New Delhi, India
2 Department of Surgery, Paras Spring Meadows Hospital, East of Kailash, New Delhi, India
|Date of Web Publication||24-Jun-2014|
Department of Pathology, Army College of Medical Sciences, Delhi Cantonment, New Delhi
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Dudani S, Maskara R. Multiple angiokeratomas of Fordyce: Lesions at an unusual site causing a diagnostic dilemma. Indian J Dermatopathol Diagn Dermatol 2014;1:46-8
|How to cite this URL:|
Dudani S, Maskara R. Multiple angiokeratomas of Fordyce: Lesions at an unusual site causing a diagnostic dilemma. Indian J Dermatopathol Diagn Dermatol [serial online] 2014 [cited 2020 Feb 18];1:46-8. Available from: http://www.ijdpdd.com/text.asp?2014/1/1/46/135200
Angiokeratomas are rare benign vascular lesions characterized by ectasia of blood vessels in the superficial dermis accompanied by secondary epidermal changes.  Four clinical types have been described, all having similar histopathological features. In males, angiokeratoma of Fordyce commonly occurs on the scrotum, thigh, groin and abdomen associated with localized venous hypertension.
We report a case of multiple angiokeratomas of the corona of the glans penis in a young male, an extremely rare location, due to which it was misdiagnosed as genital warts.
A 26-year-old healthy male patient presented with multiple, tiny, blackish, papillariform lesions on the corona of the glans penis since one year. They were soft to firm, non-tender and measuring 2-4 mm in diameter. Sexual contact with multiple heterosexual partners was positive. There was no varicocoele or inguinal lymphadenopathy. Routine laboratory investigations were normal. Venereal Disease Research Laboratory (VDRL) and human immunodeficiency virus (HIV) tests were negative. Human papillomavirus (HPV) testing was not done. The clinical diagnosis was multiple genital warts. The tumours were excised by electrocautery. Histopathology revealed a lining of stratified squamous epithelium, which showed hyperkeratosis and irregular acanthosis. The papillary dermis revealed multiple thin-walled, ectatic blood vessels containing red blood cells (RBCs), thus confirming the diagnosis of angiokeratoma [Figure 1].
The four clinical types of angiokeratoma are (i) angiokeratoma of Mibelli, characterized by papules or verrucous nodules, 3-5 mm in diameter, appearing in childhood or adolescence. (ii) Angiokeratoma of Fordyce, described by Fordyce in 1896, characterized by multiple vascular papules 2-4 mm in diameter on scrotum, thigh, groin and abdomen of adult men related to elevated venous pressure. Similar lesions are described on the vulva in females. (iii) Angiokeratoma corporis diffusum, synonymous with Fabry's disease, an X-linked genetic disorder associated with deficiency of lysosomal enzyme A galactosidase. (iv) Solitary or multiple angiokeratomas, where one or many papules, 2-10 mm in diameter arise in lower extremities of young adults. 
|Figure 1: Photomicrograph revealing a lining of stratified squamous epithelium, which shows hyperkeratosis and irregular acanthosis. The papillary dermis shows multiple, thin-walled, ectatic blood vessels containing RBCs, confirming the diagnosis of angiokeratoma|
Click here to view
Angiokeratomas of Fordyce occurring in glans penis are rare with only few reported cases. ,
Individuals present with 2-5 mm reddish-purple, round to oval papules, which can be scaly and dome shaped. Early angiokeratomas appear reddish, soft and compressible, whereas long-standing lesions are bluish, keratotic and noncompressible.  Spontaneous bleeding after slight trauma or sexual intercourse is known.
Pathogenesis of angiokeratomas is unclear but reported to be associated with localized venous hypertension  or degeneration of vascular elastic tissue.
Chronic infection with HPV has been described in vulval angiokeratomas.  A similar association with penile angiokeratomas is not known.
Our patient had some peculiarities. History of sexual contact was positive with lesions on the corona of the glans penis and no associated venous hypertension.
The morphologic similarity of angiokeratoma of Fordyce with genital warts or even malignant melanoma may pose a diagnostic dilemma to the treating dermatologist. Histopathological examination is essential to avoid any misdiagnosis.
Microscopic findings are diagnostic. The epidermis shows hyperkeratosis and variable degree of acanthosis. The papillary dermis reveals numerous, dilated and congested thin-walled blood vessels which may occasionally be thrombosed.
Treatment is usually by diathermy, electrocautery, cryosurgery or surgical excision. Laser ablation may be done for larger lesions.
| References|| |
|1.||Kirkham N. Tumours and cysts of the epidermis. In: Elder DE, editor. Lever′s Histopathology of skin. New Delhi: Wolters Kluwer/Lippincott William and Wilkins; 2009. p. 1011. |
|2.||Feramisco JD, Fournier JB, Zedek DC, Venna SS. Eruptive angiokeratomas on the glans penis. Dermatol Online J 2009;15:14. |
|3.||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;13:19. |
|4.||Erkek E, Basar MM, Bagci Y, Karaduman A, Bilen CY, Gokoz A. Fordyce angiokeratoma as clues to local venous hypertension. Arch Dermatol 2005;141:1325-6. |
|5.||Bruce DH. Angiokeratoma circumscriptum and angiokeratoma scrota. Report of a case. Arch Dermatol 1960;81:388-93. |