Causes and risk factors
The exact cause of this condition is not known. However, certain studies have shown that distention of the thrombosed or clotted blood vessel into the dermis and epidermis can lead to this condition. In some cases, a history of trauma is present. Fabry syndrome is said to be caused due to certain genetic factors.
Clinical presentation:
Formation of a papule which has a wart-like appearance is the characteristic feature of this condition. The color of the lesion is reddish black. It may vary in size. The papules lead to formation of a plaque. The papule usually range from few millimeter to an average size of 5 mm, while a plaque can be averagely of 6 mm size. These skin lesions have an irregular surface like that of a pebble. The surfaces are hard to touch. Usually these lesions are seen in solitary on the legs and can be multiple on other parts of the body. The crusty lesions can get accidentally scratched leading to bleeding. There are different types of angiokeratomas: Sporadic, Fabry syndrome, angiokeratomas of Fordyce, and angiokeratoma circumscription. Sporadic angiokeratoma is commonly seen at the age of 40 years and mostly occur as a solitary lesion. Fabry syndrome is said be an inherited disorder. It is caused due to deficiency of alpha galactosidase enzyme and ceramide trihexoside. It is commonly seen in males. These lesions are often widespread and cover various parts of the body like trunk and groin. Fever along with pain in upper and lower extremity is the associated symptom seen. Angiokeratoma circumscriptum is seen as a birthmark, particularly in females. The lesions are seen in cluster on leg and trunk. These lesions are dark in color and their size and shape keeps varying. In angiokeratoma of Fordyce, the lesions are commonly seen on scrotum, penis, and labia major of vulva in women. However, these lesions are more commonly seen in males and multiple and single lesions can be seen.
Investigations:
Diagnosis is done on the basis of the symptoms narrated by the patient and the physical examination carried out by the dermatologist. Skin biopsy along with histological examination can be done. Certain other investigations for detecting the underlying causes can also be done.
Treatment:
There is yet no effective treatment for this condition. From cosmetic point of view, excision or elctrocautery can be done. Certain other techniques like cryotherapy, laser ablation, and fulguration can be done.
Other modes of treatment:
Certain other modes of treatment can also be helpful in coping up with the disease. Taking into consideration the symptoms in a holistic way, homoeopathy can offer a good aid for the relief of the symptoms. The Ayurvedic system of medicine, which uses herbs and synthetic derivates, can also be beneficial in combating the complaints.
