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Authors: Claire Jordan Wiggins, Riyad N.H. Seervai, Medical Students, Baylor College of Medicine, Houston, Texas, USA. DermNet NZ Editor in Chief: Adjunct A/Prof Amanda Oakley, Dermatologist, Hamilton, New Zealand. Copy edited by Gus Mitchell. April 2020.
A cutaneous angiofibroma is a benign vascular neoplasm composed of dermal fibrous tissue and blood vessels.
Angiofibroma is classified by association with a genetic disorder or according to its body site .
Angiofibromas are associated with the following genetic disorders:
Angiofibromas are more commonly acquired.
Tuberous sclerosis is a neurocutaneous autosomal dominant syndrome, in which angiofibromas appear in childhood in the nasolabial folds and on the central face . Patients with tuberous sclerosis commonly develop an oral fibroma or a periungal angiofibroma (Koenen tumour) over time . The facial angiofibromas associated with tuberous sclerosis are also called adenoma sebaceum, juvenile angiofibroma, and Pringle tumour.
Angiofibromas in tuberous sclerosis
Facial angiofibromas have been reported in Birt-Hogg-Dubé syndrome, a rare genodermatosis characterised by skin and renal tumours, as well as spontaneous pneumothorax . Most of the cutaneous lesions however are fibrofolliculomas, which are abnormal growths of the hair follicles.
Multiple endocrine neoplasia type 1 is a hereditary syndrome that leads to tumours in several endocrine organs .
Angiofibromas can also be acquired and unrelated to a genetic syndrome, commonly in the form of:
A fibrous papule is characteristically found in adults as a solitary lesion usually on the nose, often clinically mistaken for a basal cell carcinoma or melanocytic naevus. It is thought to be a form of dermal naevus.
Multiple pearly penile papules occur in 10–30% of adult males on the coronal edge and sulcus. They can be mistaken for viral warts [1,2].
Angiofibromas are caused by a local overgrowth of collagen, fibroblasts, and blood vessels.
Genetic mosaicism for these genetic conditions must also be considered . What specifically triggers the development of angiofibroma is unknown.
An angiofibroma is a firm, flesh-coloured dome-shaped papule less than 5 mm in diameter. Small capillaries may be visible on the surface of the lesion.
Angiofibromas may be itchy and may also bleed. Those associated with genetic syndromes result in facial disfigurement and stigmatisation . See Psychosocial factors in dermatology.
The diagnosis of angiofibroma may be made clinically or after a skin biopsy. The histopathology of angiofibroma shows an ‘onion skin’ pattern around vessels and follicles, hyperkeratosis, and vascular proliferation .
If an underlying genetic condition is suspected, appropriate genetic screening and evaluation are required .
The differential diagnosis for angiofibroma depends on its location .
Differential diagnoses for facial lesions that can resemble angiofibromas can include:
Differential diagnoses for periungual lesions that can resemble angiofibroma can include:
Differential diagnoses for penile lesions that can resemble angiofibroma can include:
Angiofibromas are benign and do not always require removal. Options for treatment of angiofibromas include:
Multiple treatments are often necessary .
Although angiofibromas are benign, they are persistent. Angiofibromas can be removed for cosmetic or pain-related reasons. The recurrence rate for angiofibromas associated with tuberous sclerosis may be as high as 80% .
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