Answer of Dermatopathology Case 87

Angiokeratoma

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Abstract:

Angiokeratoma: a cutaneous marker of Fabry's disease. Clin Exp Dermatol. 2010 Jul;35(5):505-8. Epub 2009 Oct 19.
The initial symptoms of Fabry's disease (FD) may seem harmless and may delay its diagnosis. A survey and screening for FD were performed on men with biopsy-proven angiokeratoma and some of their relatives (n = 29). Three patients were identified. Dermatologists should be aware of this prominent early feature and investigate unexplained cutaneous vascular lesions to detect FD.

Solitary angiokeratoma of the tongue in adults. Rom J Morphol Embryol.2010;51(4):771-3.
Angiokeratomas are vascular malformations that usually appear as multiple or solitary cutaneous plaques. Several clinical variants have been described, with the same underlying histopathological lesion. Mucosal involvement, including the oral cavity, is occasionally found either as a component of the systemic variety, called angiokeratoma corporis diffusum, or associated with cutaneous lesions in more locations. Isolated oral involvement seems to be rather infrequent and only five cases have been described in adults in the world literature. We herein report another case of this rare entity affecting a 62-year-old woman in the dorsum at the tip of the tongue. This is the first report including an immunohistochemical study to discard a lymphatic origin of the tumor.

Angiokeratoma presenting as plantar verruca: a case study. J Am Podiatr Med Assoc.2010 Nov-Dec;100(6):502-4.
One of the more frequent pathologic conditions that podiatric physicians are confronted with is plantar verrucae. Plantar verrucae have been studied extensively in terms of morphological features and incidence in the population at large and in patients with human immunodeficiency virus infection. Solitary angiokeratomas can be morphologically similar to plantar verrucae, presenting as hyperkeratotic pedunculated lesions. We present a unique case study of a 40-year-old man with human immunodeficiency virus with a painful solitary angiokeratoma masquerading as plantar verrucae. The lesion demonstrated clinical signs consistent with those highlighted in the literature for verrucae, namely, showing as red and black lacunae, punctuated hyperkeratotic epidermis. We propose that solitary angiokeratomas should be an important part of a podiatric physician's differential diagnosis in the lower extremity owing to the similarity of morphological features with plantar verrucae.

Solitary angiokeratoma of the tonsillar pillar of the oral cavity. Rom J Morphol Embryol.2009;50(1):115-7.
Solitary angiokeratoma has rarely been described in oral mucosa, mainly in the tongue, where the main concern is either aesthetical or due to bleeding problems. We present a case of solitary angiokeratoma of the tonsillar pillar in a 68-year-old man. Histologically, the morphology was typical of angiokeratoma. It showed an immunohistochemical pattern in consonance with a blood vessel origin, with expression of CD31, CD34, and von Willebrand factor. The lesion did not express D2-40. No other malformation or metabolic disorder was found in the patient. The lesion was surgically removed and due to the disproportionate post-surgery bleeding, the patient was studied by the Hematology Service, and she was diagnosed as an inhibitor of Factor VIII carrier.

Angiokeratomas: an update.Dermatology.1996;193(4):275-82.
Angiokeratomas are vascular lesions which are defined histologically as one or more dilated blood vessel(s) lying directly subepidermal and showing an epidermal proliferative reaction. At the center of pathogenesis there is a capillary ectasia in the papillary dermis. The epidermal changes in all forms of angiokeratoma are secondary. The different entities causing vessel ectasia lead to the many clinical variants of angiokeratoma. Current classification distinguishes between widespread forms (angiokeratoma corporis diffusum), which is usually associated with an inborn error of metabolism, and localized forms, which include solitary angiokeratoma, Fordyce's angiokeratoma, angiokeratoma circumscriptum naeviforme and angiokeratoma of Mibelli.

Angiokeratoma of the vulva: diagnosis and review of the literature. Obstet Gynecol Surv.1989 May;44(5):339-46.
Angiokeratomas of the vulva are uncommon benign lesions. They are usually unilateral, multiple in number, and occur before the age of 50 years. Angiokeratomas are papular lesions measuring less than 1 cm in diameter and are purple in color. In most patients the lesions are asymptomatic; however, intermittent bleeding, pruritus, and pain have been reported. Histologically, hyperkeratosis, papillomatosis, acanthosis, and dilated vasculature in the papillary dermis are characteristic features. Degenerative changes in the perivascular elastic tissue is observed and may contribute to the pathogenesis of vulvar angiokeratomas. In asymptomatic patients, management need only include reassurance and follow-up observation; surgical excision, electrodesiccation, or argon laser for local removal of the lesions may be useful in symptomatic women. Clinically, infections, inflammatory lesions, vascular conditions, and epithelial tumors must be differentiated.