Dermatology - Angiokeratoma

Dermatology - Angiokeratoma 
Angiokeratoma 
An angiokeratoma is an epidermal tumor that contains capillaries and postcapillary venules, resulting in hyperkeratosis. The term "angio" refers to blood vessels, while "keratoma" indicates the presence of keratotic features.

Angiokeratomas are papules or small plaques that are dark violaceous to black in color. They are often keratotic and have a rigid texture that cannot be compressed by diascopy. Angiokeratomas can occur as either solitary lesions (solitary angiokeratoma) or in multiple numbers. Angiokeratoma of Fordyce refers to the presence of many lesions on the scrotum or vulva. Angiokeratoma of Mibelli is characterized by the presence of pink, dark red, and even black papules on the elbows, knees, and dorsa of the hands. This autosomal dominant condition is infrequent and manifests in young females. Fabry disease, depicted in the artwork, is an X-linked recessive disorder and a congenital metabolic disorder.

The lack of α-galactosidase A results in the buildup of glycosphingolipids in the dermis, heart, kidneys, and autonomic nervous system. In this form, the angiokeratomas are abundant, deep red, marked with dots, and very small (less than 1 mm), situated in the lower portion of the body: lower belly, genitalia, and buttocks, although lesions may also appear on the lips. The guys who have two identical copies of the gene also experience symptoms associated with the participation of other organ systems, such as acroparesthesias, intense pain, temporary ischemia episodes, and myocardial infarction.
Corneal opacities can occur in females who have different alleles for a particular gene. Fabry disease is an exceedingly uncommon condition.

In the majority of cases, diagnosis is based on clinical evaluation and is further validated through biopsy to exclude the presence of malignant tumors. The diagnosis of Fabry disease is established through the assessment of the patient's family history and the utilization of genetic tests. The differential diagnosis encompasses purpura, petechiae, and other metabolic illnesses. In cases when the lesions are isolated, the most significant possibility to consider is a tiny nodular or superficial spreading melanoma.

For the majority of angiokeratomas, no therapy is necessary. However, if treatment becomes necessary, treatments such as laser surgery, liquid nitrogen, and electrocoagulation can be considered.