Angiokeratoma of Fordyce: Two unusual case presentations involving lesions on the clitoris and glans penis

Introduction

Angiokeratoma of Fordyce is a benign vascular lesion characterized by dilated blood vessels in the papillary dermis, accompanied by epidermal acanthosis and hyperkeratosis. The pathogenesis is not fully understood but is thought to involve localized venous hypertension.^1–3 Clinically, angiokeratomas can mimic other conditions, including pyogenic granuloma and malignant melanoma, necessitating a thorough differential diagnosis. This report presents two cases: a 22-year-old woman with a nodular lesion on the clitoris and a 26-year-old man with violaceous papules on the glans penis, highlighting the clinical features and diagnostic challenges associated with this condition.

Case 1

A 22-year-old woman presented with a solitary, mildly tender, and prominent nodular lesion situated on the clitoris (Figure 1(a)), which had been gradually increasing in size over a period of 7 months. Initially, the lesion was small, but it had been prone to intermittent episodes of bleeding, precipitated by manipulation of the nodule. Her past medical history was limited to chronic constipation, along with a recent diagnosis of internal hemorrhoids. Her medication history was also unremarkable, with the exception of oral contraceptive consumption for the past 2 years. A comprehensive list of differential diagnoses was considered for the lesion, encompassing pyogenic granuloma, verruca vulgaris, vulvar intraepithelial neoplasia, pigmented basal cell carcinoma, condyloma acuminatum, angiokeratoma, and malignant melanoma. Given the uncertainty surrounding the diagnosis, an excisional biopsy was deemed necessary to achieve a definitive diagnosis and concurrently remove the lesion. Gross examination of the excised lesion revealed a 0.6 × 1.0 × 0.6 cm nodular mass with a soft and brown appearance. Histopathological examination (Figure 1(b) and (c)) demonstrated orthohyperkeratosis and parakeratosis, characterized by the presence of fibrin and blood, alongside focal erosions covered by a blood clot within the elevated epidermis. Additionally, dilated vascular channels with thin walls, filled with blood and thrombus, were observed extending into the deeper layers between the elongated rete ridges. Integrating the histopathological and clinical findings, we arrived at a final diagnosis of Fordyce angiokeratoma, indicating that no further treatment is necessary.OPEN IN VIEWER

Case 2

A 26-year-old man presented with a 5-month history of asymptomatic, slightly elevated, violaceous papules localized to the glans penis (Figure 2(a)). He was referred for further evaluation following urologic consultation that initially suspected the lesions to be condylomata acuminata. A thorough urologic examination was performed, which returned normal findings, with no evidence of varicocele or inguinal hernia. The patient’s medical history was otherwise unremarkable.OPEN IN VIEWER

Clinical assessment of the lesions prompted consideration of several differential diagnoses beyond genital warts, including lichen planus, psoriasis, and vascular tumors. To achieve a definitive diagnosis, a punch biopsy was conducted. Histopathological analysis (Figure 2(b) and (c)) revealed hyperkeratosis and parakeratosis of the epidermis, along with dilated vascular structures within the papillary dermis, lined by non-dysplastic endothelial cells and containing thrombi. These histological features were consistent with a diagnosis of angiokeratoma.

Discussion

Angiokeratoma of Fordyce is a benign vascular lesion characterized by dilated blood vessels in the papillary dermis, along with epidermal acanthosis and hyperkeratosis. ¹ These lesions appear as papules, which can be few or numerous, and vary in color from red to blue, purple, or black, measuring between 0.5 and 5 mm ² They are predominantly located in the genital region and are generally asymptomatic; however, they may occasionally present with bleeding, underscoring the importance of clinical awareness. These lesions are more common in males but can also occur in females, especially in older age groups. ¹ The exact cause of angiokeratoma of Fordyce is not fully understood, but localized venous hypertension is believed to play a significant role. ³ In males, lesions typically appear symmetrically in the scrotal area, while in females, they mainly affect the vulva, particularly the labia majora. ⁴ Occurrences in the clitoris and glans penis are rare, with only a few documented cases of clitoral angiokeratoma in patients aged 14 to 78. The disease can manifest variably, with some lesions developing gradually and others progressing rapidly, presenting symptoms that range from asymptomatic to painful and necrotic.^1–3,5,6 Angiokeratomas on the penis often appear as multiple lesions along the coronal rim of the glans, though solitary lesions can occur. ⁷ Management typically involves observation, as these lesions are benign and often asymptomatic. For cosmetically undesirable or symptomatic lesions, treatment options include cryotherapy, electrocoagulation, laser therapy, sclerotherapy, and surgical excision. ⁸ Despite their infrequency, it is clinically prudent to consider these presentations alongside other critical differential diagnoses, such as melanoma, as thrombosed angiokeratomas can mimic the appearance of melanoma. ⁴ Management of angiokeratomas is generally simpler than that of melanoma. Clinicians must carefully evaluate these lesions to avoid misdiagnoses and ensure proper management of any potential complications.

Conclusion

Angiokeratoma of Fordyce, while generally benign and often asymptomatic, poses diagnostic challenges due to its potential to resemble more serious dermatological conditions. The cases presented herein illustrate the critical need for comprehensive clinical evaluation and histopathological analysis to achieve an accurate diagnosis. Given the infrequency of these lesions in the clitoris and glans penis, heightened awareness among dermatologists is essential for differentiating them from other pathologies, particularly malignancies. Management strategies may range from observation to intervention based on symptomatology and cosmetic considerations. Ongoing documentation and investigation of such cases are imperative for enhancing our understanding and guiding effective management of angiokeratomas in the genital region.