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The term angiokeratoma is applied to several distinct conditions with vascular skin lesions, the histology of which shows superficial vascular ectasia and overlying hyperkeratosis.

1

The angiokeratomas should be regarded as capillary vascular malformation rather than hemangiomas.

1

Apart from angiokeratoma corporis diffusum (Fabry), which is a disorder of phospholipid metabolism, at least four types of purely cutaneous angiokeratoma are known: the Mibelli type, the Fordyce type, angiokeratoma circumscriptum and solitary and multiple angiokeratoma (Imperial and Helwig). We present here a case of angiokeratoma circumscriptum and a brief review of the literature.

Blackwell Publishing, Ltd.Oxford, UKIJDInternational Journal of Dermatology1365-4632Blackwell Publishing Ltd, 200445

Case report

Angiokeratoma circumscriptum

Mittal

et a.CAMEO

Angiokeratoma circumscriptum: a case report and review of the literature

Rachna Mittal,

MD

, Asok Aggarwal,

MD

, and Govind Srivastava,

MD

From the Skin Institute and School of Dermatology, New Delhi, India

Correspondence

Rachna Mittal Skin Institute and School of Dermatology N block, Greater Kailash-I New Delhi-110 048 India E-mail: pnbsisd@nde.vsnl.net.in

Introduction

The term angiokeratoma is derived from three Greek wordsmeaning vessels, horn and tumor, respectively.

2

These arerare verrucous vascular lesions that appear as dark redpapules and plaques arranged either discretely or in clusters.The exact mechanism for their development is not known,although congenital cause, pregnancy, chilblains, trauma andtissue asphyxia are given as causal factors.

3

These lesions, asImperial and Helwig

2

wrote, are not true angiomas but aremerely telangiectasias of preexisting vessels. The basic path-ological process is dilatation of the papillary capillaries withresultant secondary epidermal changes of hyperkeratosis andacanthosis, as a means of preventing further dilatation andrupture.

2

Angiokeratoma circumscriptum is one of the fivedisorders in the group of angiokeratomas. The various typesdiffer clinically from each other by location and morphologybut are histologically similar (Table 1).

Case Report

A 25-year-old man had, since birth, several eruptionsarranged linearly along the external aspect of his rightcalf to his ankle. He reported episodes of bleeding after minortrauma. No symptoms suggesting hematuria, melena orhemoptysis were reported during the course of the disease.General physical examination revealed normal vital signs.Legs were equal in length and circumference. Vision and aneurological evaluation were normal. The lesions consisted ofseveral asymptomatic vascular plaques which had graduallyincreased in number and become hyperkeratotic and verru-cous (Figs 1 and 2). No pulsation was detected by palpation

of the lesions. They were barely compressible and did notblanch. Skin biopsy revealed a compact type of hyperkeratosiswith slight papillomatosis and a thin granular layer. Papillarydermis showed several dilated capillaries containing manyerythrocytes (Figs 3 and 4). There were also areas of hemor-rhage in the reticular dermis near the eccrine sweat glands.Based on clinical and histopathological features, a diagnosisof angiokeratoma circumscriptum was made.

Discussion

Since the first description of angiokeratomas in 1890

4,5

theselesions have been recorded as a manifestation of a number ofdifferent diseases. Fabry

6

in the year 1915 classified angiok-eratomas into two types: the diffuse and the circumscribed. In

Figure 1 Lesions on the lateral and ventral aspect of the right leg and foot

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Mittal

et al.

subsequent years, a variety of circumscribed cases weredescribed under different names including congenital unilat-eral nevus of angiokeratomatous structure,

7

nevus kerato-angiomatosis,

8

nevus vascularis,

9

angioma verrucosum

10

andangiokeratoma corporis neviforme.

11

These divergent opinions prompted Dammert

12

to reviewthe earlier case reports and proposed angiokeratosis nevi-forme as a more suitable name. Later on certain generaliza-tions were made by Lynch and Kosanovich

13

on the basis ofapproximately 50 cases. However, some of these lesionsdescribed as angiokeratoma circumscriptum in the literaturewere, probably, verrucous hemangioma

13,14

or lymphangi-oma circumsriptum.

12

In 1967 Imperial and Helwig

15

intro-duced the term verrucous hemangioma and distinguished itfrom angiokeratoma and its variants.

Angiokeratoma circumscriptum is a rare vascular malfor-mation of the papillary dermis evolving through stages. Thelesions are typically situated unilaterally on the lower legs orfeet, but can occur on the thighs, buttocks or occasionallyelsewhere.

1

They are deep red to blue-black in color and tend to take azosteriform configuration. The lesions become increasinglystudded with warty keratotic papules or nodules. There is notendency to spontaneous improvement.

1

If thrombosis occurs within angiokeratoma, malignantmelanoma may be mimicked clinically.

16

Histologically, angiokeratoma circumscriptum appears asdilated dermal papillary capillaries drained by dilated venules.The overlying epidermis shows a variable degree of acantho-sis, papillomatosis and compact hyperkeratosis. There is no

Table 1 Comparison of five clinical types of angiokeratoma

Sub. no. Type Description Age Sex Hereditary Comments

1.* Angiokeratoma Purple, red, dark Before Predominantly Yes Associated with heartcorporis diffusum blue, slightly puberty in males but also and kidney disease.(Fabrys disease raised papules, occurs in females Other features include1898)23 13 mm, scattered recurrent episodes of

over trunk and fever and paresthesiasproximal beginning in childhoodextremities and corneal opacities24,25

2. Angiokeratoma Warty, vascular Childhood to Both sexes but No Associated withMibelli (1889)26 lesions (25 mm) adolescence predominantly chilblains

usually present on in young girlsthe dorsa of handsand feet

3. Angiokeratoma Warty, vascular Adulthood Male No Half of the casesFordyce (1895)27 lesions (15 mm) or second are associated

limited to the to third with increasedscrotum decades venous pressure, such as varicocele

4. Angiokeratoma Lesions usually At birth Both No circumscriptum occur unilat-(1915)6 erally on a

lower extremity.They appear ina zosteriformfashion and maylater becomelarge and warty

5. Solitary and Lesions similar to Second Both No Frequentlymultiple Mibelli type, smaller to fourth mistakenangiokeratoma than angiokeratoma decades for nevus or(1965)1 circumscriptum, occur malignant

on any part of body melanomabut common onlower extremities

*Systemic glycolipidosis owing to deficiency of ceramide trihexosidase.28 But lesions similar to those of Fabrys have also been seen in fucocidosis and sialidosis and also in phenotype, suggestive of mucopolysacharidosis.29 To date, only three cases of widespread angiokeratomas with no evidence of any metabolic disease have been reported in the literature.2931

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Mittal

et al. Angiokeratoma circumscriptum

Case report

involvement of the deep dermis and hypodermis. The papil-lary vessels may form large lacunae in the papillary area of thedermis and may also be thrombosed.

1,2,17

The elongated reteridges may partially or completely encase the vascular lacunae

forming the so-called blood cyst.

18,19

Lesions with deeperangiomatous elements have been reported under verrucoushemangiomas. The differential diagnosis includes verrucoushemangioma, Cobb syndrome, angioma serpiginosum, lym-phangioma circumscriptum, verrucae, and certain tumors,including melanoma.

Verrucous hemangioma is a congenital, localized, vascularmalformation, histologically characterized by dilated capil-laries and large cavernous, endothelial-lined, blood-filledspaces extending well into the reticular dermis and subcuta-neous fat and with hyperkeratosis.

17

Certain unique featureseasily distinguish angiokeratoma circumscriptum, which isan acquired dermatosis, histologically characterized bydilatation of the vessels of the papillary dermis, withoutinvolvement of the deep dermis and hypodermis and withhyperkeratosis. It is classified among the hypertrophic angio-producing tumors (telangectasias), characterized by a singlevascular dilatation without cellular proliferation, while, ver-rucous hemangioma is considered among the angio-producing

Figure 2 Details of the lesions. Linear hyperkeratotic plaques with smaller satellite lesions and purpuric patches

Figure 3 Epidermis shows hyperkeratosis and slight papillomatosis. Papillary dermis shows several dilated capillaries (hematoxylin and eosin, 40)

Figure 4 Details of the dermis show dilated capillaries containing erythrocytes (hematoxylin and eosin, 100)

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hyperplastic tumors, characterized by proliferation of mesen-chymal cells tending to the formation of capillaries.

17,20

Lymphangioma circumscriptum is differentiated by theproteinaceous contents present in its vascular spaces but iferythrocytes have spilled into the spaces the differentiationmay be difficult. The clinical appearance of clear vesicles maysometimes help.

Cobb syndrome is an uncommon congenital vascularnevus with a dermatomal disposition on the trunk associatedwith an underlying meningospinal angioma, which ofteneventuates in a distal flaccid paraplegia.

21

Angioma serpiginosum presents as small violaceous-redpuncta with a serpiginous distribution, which lacks the hyper-keratotic surface.

22

Diagnosis is mainly histopathological, although a correctclinical suspicion is important in order to obtain an adequatedeep biopsy specimen for ruling out verrucous hemangioma.Angiokeratoma circumscriptum does not regress spontane-ously. Local excision or electrocautery are the best treatmentsfor smaller lesions, while the larger lesions require wide, deepexcision and grafting. It responds to the common means of phys-ical therapy (diathermocoagulation, cryotherapy, Argon laser)and recurrences are rare as opposed to verrucous hemangioma.

In our patient we performed a deep excision of the wartylesions with the help of a electric cautery cutting machine.Unfortunately, the patient did not return for the follow up.

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