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Angiokeratoma circumscriptum naeviforme: A case report of a rare disease

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Angiokeratoma circumscriptum naeviforme: A case report of a rare disease
Sudip Kumar Ghosh MD DNB1, Debabrata Bandyopadhyay MD1, Loknath Ghoshal MD1, Susmit Haldar MD DNB2
Dermatology Online Journal 17 (9): 11

1. Department of Dermatology, Venereology, & Leprosy, R G Kar Medical College, Kolkata, India.
2. Calcutta Skin Institute, Kolkata, India


Angiokeratomas are relatively rare vascular lesions characterized by ectasias of the papillary dermal blood vessels with secondary epidermal changes in the form of acanthosis and /or hyperkeratosis. Amongst the several variants of angiokeratomas, angiokeratoma circumscriptum is the least common one. Angiokeratoma circumscriptum naeviforme (ACN) is a still rarer type of angiokeratoma, which is typically seen at birth. We report here a case of linear, unilateral, verrucous plaques on the leg of a young man, diagnosed as ACN.


Angiokeratomas are relatively rare vascular lesions characterized by dilatations of the papillary dermal blood vessels with secondary epidermal changes of acanthosis and /or hyperkeratosis [1]. The precise mechanism for their development is unknown [2]. Based on the clinical presentation and evolution, the current classification of angiokeratomas includes solitary and multiple angiokeratoma, angiokeratoma of Fordyce, angiokeratoma of Mibelli, angiokeratoma circumscriptum, and angiokeratoma corporis diffusum [1, 2, 3]. All the variants have identical histopathological features [2, 3].

Angiokeratoma circumscriptum, the rarest variant [1, 3], is clinically characterized by dark red to blue-black nodules or plaques that are typically situated unilaterally on the lower legs or feet. It may also occur on the thighs, buttocks, and occasionally elsewhere in the body. The lesions often become keratotic and show no tendency for spontaneous remission [2]. Angiokeratoma circumscriptum naeviforme (ACN) is a still rarer variant of angiokeratoma, classically present since birth [4].

We report here such a case of ACN, in the form of linear unilateral verrucous plaques on the leg of a young man.

Case history

A 20-year-old man presented to us with a linear eruption on his left leg, which was present since his birth. The cutaneous lesions gradually became more extensive and hyperkeratotic with age. However, the lesions have remained static over the past few years. He gave a history of occasional pain and several episodes of mild bleeding from the lesions after trivial trauma. There were no other systemic symptoms or history of external bleeding from any other site. There was no history of similar disease in his family.

Figure 1Figure 2
Figure 1. (a) Discrete as well as confluent hyperkeratotic plaques in linear arrays. An area of traumatic ulceration is also visible. (b and c) Close-up view of lesions showing a verrucous surface

Figure 2. Compact hyperkeratosis and acanthosis with slight papillomatosis. Dilated vascular spaces containing erythrocytes in the papillary dermis. (H&E, x100)

Cutaneous examination revealed several discrete and confluent verrucous plaques of varying sizes, arranged linearly along the extensor aspect of the upper third of his left leg, extending to the ankle (Figure 1). The lesions were firm, non-compressible, and non-tender. No pulsation was felt on lesional palpation and no bruit was heard on auscultation. An area of traumatic ulceration was also visible over the lesion. There was no regional lymphadenopathy. His legs were equal in length and circumference. Systemic examination was normal. Routine laboratory investigations including complete hemogram and biochemistry panel were non-contributory. An X-ray of the legs showed no bony abnormalities. Incisional biopsies were taken from multiple sites.

Histopathological examination of all the biopsy specimens showed similar features of compact hyperkeratosis, acanthosis, and slight papillomatosis. The papillary dermis showed several dilated capillaries containing erythrocytes (Figure 2). No vascular ectasia was found in the reticular dermis or in the subcutis.

Based on the clinical and histopathological features, a diagnosis of angiokeratoma circumscriptum naeviforme was made.


The lesions of ACN are typically present since birth as linear, unilateral, hyperkeratotic plaques composed of confluent keratotic papules [4]. However, the nevus may not be perceptible until childhood or even adulthood [5]. Nevoid malformation of the underlying vasculature is thought to be the fundamental cause of ACN [4]. The lesions may vary from a few centimeters to very large in size. In some cases, the lesions initially present as multiple reddish macules, clinically resembling nevus flammeus. Within a few years, they develop into plaques with a hyperkeratotic verrucous surface [5].

The lesion may be related to an underlying vascular malformation and/or atrophy or hypertrophy of the local soft tissue and bone. The sites of predilection on the skin are the gluteal regions and the thighs. The coexistence of ACN with other types of angiokeratoma and other vascular malformations, including angiokeratomas of Fordyce, Cobb syndrome, Klippel-Trenaunay syndrome, nevus flammeus, cavernous hemangioma, and traumatic arteriovenous fistula has been reported in the literature [5]. We did not find any such coexistence in our patient.

Our main consideration in the differential diagnosis of the present case was verrucous hemangioma. Verrucous hemangioma is a congenital, localized, vascular malformation, histopathologically characterized by dilated capillaries and large endothelial-lined, blood-filled spaces extending well into the reticular dermis and subcutaneous tissue with an overlying hyperkeratotic epidermis [6]. On the other hand, angiokeratoma, as in our case, does not show the deep angiomatous component of verrucous hemangioma [4, 5].

The other clinical diagnosis considered in the present case was verrucous epidermal nevus. However, histopathological features could easily differentiate it from ACN.

Small lesions of angiokeratoma may be treated by diathermy, curettage and electrocautery, cryosurgery, and surgical excision. Larger lesions may require laser ablation (carbon dioxide or argon laser) because they often are too extensive for surgical excision [5]. Owing to the lack of laser at our institution, we referred our patient to our colleagues in the plastic surgery department for further management.

We seek to emphasize by this report that, in spite of its rarity, a diagnosis of ACN should be considered for any linear verrucous growth present since birth.


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