Angiokeratoma of Fordyce is a special case of vascular dermatosis with the most frequent localization on the skin of the scrotum. The neoplasms are single or multiple, resemble small dark nodules of a purple or reddish hue with a scaly surface. The pathology is asymptomatic, but itching or soreness may be present, which increases after sexual contact or irritation. The diagnosis is confirmed by complaints, anamnesis, examination (including dermatoscopy) and biopsy results. There is no specific treatment, with bleeding and frequent infection, surgical removal is possible.
ICD 10
D18.0 I78 L81.7
Meaning
In 1896, John Addison Fordyce, an American dermatologist, first described angiokeratomas on the scrotum of a 60-year-old man with concomitant varicocele. The term “angiokeratoma” is of Greek origin and means “keratinizing vascular tumor”, although the formation is not a true tumor. In fact, it is vascular dermatosis with dilated capillaries at the surface of the skin and hyperkeratosis. The formation is benign, has no tendency to malignancy. The exact prevalence is unknown, pathology is registered less frequently in women. The probability of angiokeratoma formation increases with age: from 0.6% in 16-20 years to 16.6% in 70 years and older.
Causes
The reasons for the formation of angiokeratoma of Fordyce are debatable. External manifestations are caused by ectasia (expansion) of small superficial vessels, which gives the nodules color, and hyperkeratosis (peeling) of the overlying epidermis. It is noted that the probability of pathology is closely related to a decrease in vascular tone and a local increase in venous pressure, the causes of which may be the following conditions:
- Diseases of the pelvic organs. These include varicose veins of the spermatic cord and hydrocele in men, varicose veins of the vulva in women, inguinal hernia, tumors of the genitourinary system (prostate cancer, bladder, testicular appendages). The occurrence and aggravation of local venous hypertension is also promoted by overweight, constipation, straining during urination, and a persistent cough.
- Traumatization of the genitals. Accidental damage to the genitourinary organs, including iatrogenic injuries and operations on the penis or scrotum, can complicate venous outflow. Hypothermia, radiation therapy for pelvic tumors in the anamnesis may have an adverse effect.
- Endocrine and metabolic disorders. There is a clear relationship between hormonal changes and the development of scrotal angiokeratoma. Puberty, andropause, hormone therapy, metabolic disorders, unbalanced diet, smoking are risk factors that increase the likelihood of vascular formations.
Pathogenesis
It is assumed that the pathogenesis of angiokeratoma of Fordyce is associated with degenerative changes in the smooth muscle fibers of the fleshy shell and elastic fibers of the skin of the scrotum caused by aging. Dystrophic processes in soft tissues contribute to the loss of vascular wall tone and dilation of blood vessels. Long-term high blood pressure (localized venous hypertension) it further aggravates damage to the walls of papillary capillaries, their dilation and deformation. In andrology, there is evidence of regression of scrotal angiokeratoma after elimination of varicocele. However, the role of concomitant local venous hypertension remains uncertain and requires further study.
Symptoms
Pathology is represented by multiple or single vascular nodules of dark red or deep purple color, resembling a scattering of beads. Initially, small bright reddish papules appear in the type of spot rash. Most patients do not pay attention to them, since there are no other clinical manifestations. The diameter of the elements is variable: from 2 to 5 mm. Typical localization is the skin of the scrotum, penis, sometimes the inner surface of the thighs, lower abdomen. Fordyce angiokeratomas are much less common in elderly women in the vulva, clitoris or labia majora.
The density of neoplasms and the intensity of peeling depend on the duration of the existence of vascular dermatosis and the age of the patient: fresh rashes are red, soft to the touch, with a shiny surface, and old ones – from dark blue to black and purple, rough, dense, with multiple scales, they are more common in older men. Concomitant redness of the skin is observed in 50% of patients, even with an asymptomatic course of dermatosis. In some cases, there is a tendency to the proliferation of pathological foci due to the fusion of telangiectasias, sometimes angiokeratomas occupy the entire surface of the scrotum skin. Lymph nodes are not enlarged, there is no discharge from the urethra. Often the only reason to consult a doctor is false suspicions of a sexually transmitted disease or an oncological process.
Complications
With irritation, compression, sexual contact, papules can bleed, but massive bleeding is not typical for scrotal angiokeratomas. Non-compliance with the rules of intimate hygiene, weakening of the immune system, constant combing of itchy elements lead to secondary infection. Some authors attribute psychological discomfort to complications of genital angiokeratoma.
Diagnostics
The primary diagnosis is carried out on the basis of complaints and inspection data. Patients with symptoms of Fordyce angiokeratoma in the scrotum or penis often turn to a urologist or andrologist. This is not entirely correct, since the management tactics for vascular dermatosis is determined by a dermatovenerologist, in doubtful cases (suspicion of malignancy), the patient is referred to a dermatooncologist for consultation. The diagnostic algorithm includes the following:
- Dermatoscopy. To distinguish a vascular lesion from a melanocytic formation (melanoma) or other malignant tumor, a dermatoscopy is performed. Angiokeratoma is characterized by large, well-delimited round-oval lacunae of red-black color. The fabric in the spaces between them has a pale red, pink, purple or bluish hue. A blue-white veil indicates superficial hyperkeratosis.
- Morphological verification. A biopsy with histological examination is performed for the final verification of the diagnosis. Under the microscope, when staining the drug with hemotoxylin and eosin, moderate hyperkeratosis, acanthosis with epidermal hyperplasia, numerous enlarged overloaded capillaries with elements of thrombosis, recanalization (formation of additional vascular collaterals) are visible.
There are a number of pathologies that mimic vascular dermatosis. For differential diagnosis, Fabry syndrome, hemangioma, infected genital warts, pyogenic granuloma, melanocytic nevi are significant. Fordyce angiokeratoma may resemble melanoma of the skin, which is caused by pigmentation on the background of intraepidermal hemorrhages and thrombosis, squamous cell carcinoma of the penis or keratoacanthoma. Tumor-like keratosis (a synonym for keratoacanthoma) sometimes tends to malignancy, so all incomprehensible neoplasms, even if seemingly harmless, must be shown to a doctor. To establish a final diagnosis, consultations with an oncologist, a geneticist (to exclude Fabry syndrome), a urologist may be required.
Treatment
In the absence of complaints and confidence in the benign course, no active actions need to be taken. There is no etiotropic therapy, since the causes of angiokeratoma formation have not yet been clarified. In case of bleeding, inflammation, itching or concern of the patient with a cosmetic defect, several types of surgical care are available:
- Surgical excision. It is possible with single angiokeratomas, with a large area of the injured surface there is a risk of relapse. Wide excision within healthy tissue is the operation of choice if a malignant process is suspected. Without fail, a piece of material is sent for morphological examination.
- Cryotherapy. The method involves the destruction of neoplasms using liquid nitrogen. Cryodestruction is suitable for the destruction of multiple elements. The disadvantages include areas of hypopigmentation and scarring after the intervention. Treatment with liquid nitrogen is carried out under local anesthesia, painful sensations after the procedure may persist for several days.
- Electroexcision. The point effect of electric current of various frequencies is used to remove small formations. The nodule is heated by thermal energy, which leads to protein coagulation. If there are not too many elements, one electrocoagulation session is enough. After the procedure, a small burn remains, which heals within a few days. There is no bleeding, as there is a “sealing” of the vessels.
- Laser destruction. Laser removal of Fordyce angiokeratoma is one of the modern and minimally invasive methods. Due to the accuracy of the beam guidance, nearby tissues do not suffer. After laser therapy, a barely noticeable scar remains, pain is expressed slightly, relapses occur rarely. Long-term rehabilitation is not required, and the laser exposure itself prevents the activation of microbial flora. Rapid tissue regeneration and restoration of blood microcirculation (which is important to prevent the re-formation of angiokeratomas) allow us to recommend laser destruction as a method of choice.
Prognosis and prevention
With confirmed good quality, the prognosis for life is favorable. No special preventive measures have been developed, but experts recommend timely preventive examination. Angiokeratomas do not cause harm, the possibility of infection of the partner is excluded. The danger may lie in the erroneous non-recognition of melanoma of the skin, which, being an aggressive malignant tumor, quickly metastasizes. The progression of the oncological process in the absence of treatment leads to fatal consequences. Men with diseases that violate the normal act of urination need adequate therapy, since straining provokes local venous hypertension.