Angiokeratoma Circumscriptum

Abstract

Angiokeratoma circumscriptum is one of five types in the group of the angiokeratomas. These lesions are usually present at birth and are not related with systemic diseases. Angiokeratoma circumscriptums are hyperkeratotic papules or nodules occurring frequently unilaterally on the leg. These lesions are very rare and very few cases have been reported since its first description. It is of particular clinical importance because of its morphologic similarities to malignant skin tumors such as malignant melanoma or pigmented basal cell carcinoma. Therefore, this rare but significant lesion must be recognized by physician and surgeons who manage skin tumors.

Late onset palmar angiokeratoma circumscriptum: An unusual presentation

“…Among these five types of LAK, solitary AK is the most common while AC is the least frequent. [2] AC presents as unilateral localized hyperkeratotic vascular skin lesions, affecting females more often than males usually in infancy or early childhood. Late onset, as in this case has only been rarely reported.…”

“…LAK have a prevalence of 0.16%. [2] Lesions of AC are typically situated unilaterally on the lower legs or feet, but can occur on the thighs, buttocks, or occasionally elsewhere. [3] The age of onset of AC is generally at birth.…”

Vascular Skin Lesions in Children: A Review of Laser Surgical and Medical Treatments

“…Angiokeratomas are characterized histologically by dilations of superficial dermal vessels and hyperkeratosis of the overlying epidermis . Angiokeratoma circumscriptum is a rare disorder, often presenting at birth, consisting of solitary or multiple angiokeratomas, usually occurring unilaterally on the lower extremities . Angiokeratoma corporis diffusum, or Fabry disease, is an X‐linked inherited disorder caused by a deficiency of the lysosomal enzyme alpha‐galactosidase.…”

Angiokeratoma circumscriptum of the buccal mucosa: a case report and literature review

“…In the majority of cases, it is also associated with epidermal reactions such as acanthosis and/or hyperkeratosis1. This disorder can be classified into five types: (1) a generalized systemic form (angiokeratoma corporis diffusum of Fabry); (2) a bilateral form occurring on the dorsal surfaces of the fingers and toes (angiokeratoma of Mibelli); (3) a localized scrotal form (angiokeratoma of Fordyce); (4) a solitary papular angiokeratoma; and (5) a multiple papular and plaque-like form (angiokeratoma circumscriptum)2,3. Although the clinical characteristics are different, common histological features include hyperkeratosis of the epidermis and dilated vascular spaces with or without organizing thrombi in the papillary dermis of cutaneous lesions4,5.…”

“…Although the clinical characteristics are different, common histological features include hyperkeratosis of the epidermis and dilated vascular spaces with or without organizing thrombi in the papillary dermis of cutaneous lesions4,5. Among the five types of angiokeratomas, solitary angiokeratoma is the most common, and angiokeratoma circumscriptum is the least frequent2.…”