Vascular Malformations, Haemangiomas and other Vascular Tumours

Latest update 16/06/09

Introduction

In 1982 Mulliken and Glowacki proposed the following biologic classification of vascular malformations that has since gained wide acceptance:

• Haemangiomas - are vascular tumours that exhibit endothelial hyperplasia. The majority present in infants / early childhood, with some being visible at birth. Initial growth of these tumours can be rapid. Lesions eventually start a process of gradual involution
• Vascular malformations - are structural faults that arise in utero. Endothelial cell proliferation is normal. The majoritry are visible at birth and remain through life. Lesions are often soft / cystic and compressible, and grow proportionally with the child. Vascular malformations are divided into arterial, venous, arteriovenous, lymphatic - either local or diffuse, and capillary (e.g port-wine stain)

Although many of these lesions are isoltaed phenomena it is important to be aware of a number of syndromes such as the Struge-Weber syndrome that can be assocaited with these malformations

For the purpose of this chapter the different vascular malformations and haemangiomas have been grouped together along with other vascular tumours so as to aid GPs approach these conditions in a practical manor:

• Group I: Common haemangioma's and vascular malformations of infancy & childhood (e.g. strawberry naevus; salmon patch; port wine stain)
• Group II: Acquired benign angioma & angiokeratoma
  (e.g. simple angioma, Campbell de Morgan Spot, Angiokeratoma of Fordyce, Anderson-Fabrey’s Disease)
• Group III: Other vascular lesions – common
  (e.g. spider naevus, pyogenic granuloma, venous lake)
• Group IV: Other vascular lesions – uncommon / rare
  (e.g. glomus tumours, blue rubber bleb naevus syndrome, angiolymphoid hyperplasia, angioma serpiginosum, lymphangioma circumscriptum, naevus anaemicus, Kaposi’s sarcoma, angiosarcoma)

N.B. For other vasuclar disorders such as conditions associated with marked telangiectasia please refer to the vascular section on the general dermatology image atlas   

Group I: Common haemangioma's and vascular malformations of infancy & childhood

Diagnosis: Infantile haemangioma (syn. capillary haemangioma)

Aetiology

• Infantile haemangiomas are benign proliferations of vascular lesions

Key diagnostic features

• The most c ommon presentation is of a rapidly growing, obviously vascular swelling in a baby a few days or a few weeks old, although some are present at birth
• Increased risk if prenatal chorionic villus sampling (CVS) performed
• Often single but multiple lesions can develop (more than 10 is uncommon)
• Distribution - 60% of the lesions occur on the head and neck, other common sites include the buttocks and perianal area
• Tumours can be superficial or deep, although many have features of both and are termed mixed infantile haemangioma
• Lesions with a significant superficial element are commonly known as ‘Strawberry Naevi’ and are red, relatively soft, and somewhat irregular in outline
• The deeper type are more blue and rather diffuse swellings
• Apart from the occasional lesion that is fully developed at birth (congenital infantile haemangioma), infantile superficial haemangioma grow to reach a maximum size at around 6-12 months. Deep lesions can grow for longer to become very large causing anatomical distortion
• Almost 100% undergo spontaneous regression, up to 95% of which will lead to complete / almost complete resolution. 90% of lesions resolve by the age of 9

Additional notes

• Management – The majority of lesions need no treatment. Regular monitoring in primary care may reassure parents. Photographs at 6-12 month intervals are very useful
• The following lesions need referral for further management:
  • Involving aesthetically important areas such as the face - refer early
  • Interfering with breathing, feeding, or vision – refer urgently. Amblyopia can occur within 10 days of lesions interfering with vision
  • Anywhere on the body if complicated by bleeding, ulceration or causing functional impairment
  • Lesions overlying the lumbosacral area: up to 50% of such cases are associated with significant problems such as tethering or compression of the spinal cord, and external genital abnormailites. MRI imaging should be perfomed in such patients
  • Very rarely a child develops visceral haemangiomas. This primarily, although not exclusively, occurs in patients born with a very large number of lesions (military haemangiomatosis of infancy)
  • Kasabach-Merritt syndrome ( consumption coagulopath y) is an uncommon complication, mainly in larger lesions.
• Referral should be to a dermatology department with laser facilities

Figure 1 – Strawberry naevus of axillary region

Diagnosis: Salmon patch (syn. naevus simplex; stork bite if involves the forehead)

Key diagnostic features

• Affects almost 50% of neonates
• Distribution – Nape of neck, occiput and face are the most common sites
• Irregular, dull pink-red macular area
• Lesions on the face often resolve in the first year of life, whereas 50% of nuchal lesions persist into adult life
• No treatment is needed

Figure 2 – Stork Bite

Diagnosis: Port-wine stain (syn. naevus flammeus)

Aetiology

• A vascular malformation of developmental origin with ectasia of superficial dermal capillaries

Key diagnostic features

• Almost always present at birth
• Distribution – Face > upper trunk. Any site can be affected
• Pink to deep red / purple patches
• Often unilateral with a distinct cut off
• Lesions tend to persist, darken and thicken with age

Additional notes

• Facial lesions need early referral for the following reasons:
  • The possibility of the Sturge-Weber syndrome (see below)
  • The cosmetic impact of such lesions can be substantial – referral to a dermatology department with laser facilities is appropriate
  • Lesions on or near the eyelid can be associated with glaucoma – where the face is affected both above and below the eye the risk of glaucoma is particularly high. 40% of the cases of glaucoma arise in infancy, but glaucoma can also arise in adults and those at risk need early assessment and long-term follow up with an ophthalmologist
• The Sturge-Weber Syndrome
  • The usual cutaneous finding is a unilateral port-wine stain, involving roughly the areas served by the ophthalmic and maxillary divisions of the trigeminal nerve
  • Epilepsy occurs in up to 90% of cases – once seizures start neurological deterioration with mental retardation can be rapid
  • Patients without seizures fare much better
  • Glaucoma and other eye complications arise in 50-60% of all cases
  • Early referral is indicated
• The Klippel-Trenaunay Syndrome – A rare disorder with a port-wine stain on the limb, soft tissue swelling and increased limb size. The condition is associated with many other developmental defects and high rates of DVT and pulmonary embolism

Figure 3 – Port-Wine Stain and Sturge-Weber Syndome

Group II: Acquired benign angioma & angiokeratoma

Diagnosis: Simple angioma

Key diagnostic features

• A red-blue-purple nodules
• Can occur anywhere on the body surface
• Clinical diagnosis usually straight forward
• Dermoscopy reveals multiple well-defined lacunes

	Figure 1 – Simple angioma
	Figure 2 – Dermoscopic view of figure 1 showing the classical red lacunes
	Figure 3 – Simple angioma of the scalp
	Figure 4 – Simple angioma At first glance this lesion could appear suspicious, however the lesion is compressible and the dermoscopic image is reassuring
	Figure 5 – Dermoscopic view of figure 4 showing red/purple lacunes
	Figure 6 - Simple angioma
	Figure 7 - Dermoscopic appearance of figure 6
	Figure 8 - Simple Angioma This elderly patient gave a history of a relatively small blue lesion on her neck for several decades, which then unexpectedly grew over a period of two months. Due to the history the lesion was excised. Histology confirmed the diagnosis
	Figure 9 - Dermoscopic appearance of figure 8 The blue colour of the angioma suggests a much greater venous aspect to the lesion

Diagnosis: ‘Cherry’ angioma (syn. Campbell de Morgan spot)

Key diagnostic features

• Small red > purple papules
• Often multiple lesions - numbers increase with age
• Can occur on any part of the body

Figure 10 – Mulitple cherry angioma

Diagnosis: Angiokeratoma

Key diagnostic features

• The term angiokeratoma is applied to a number of conditions that share the clinical presentation of hyperkeratotic vascular lesions, which tend to start off bright red and darken with age
• Most forms are acquired
• Lesions often multiple but solitary lesions can occur
• Solitary angiokeratoma are most commonly found on the lower extremities and although harmless, they can darken quite quickly giving the patient cause for concern
• Angiokeratoma of Fordyce is the most common form and presents with increasing frequency with age – it is extremely common in older men and women. Small and bright red papules that enlarge and get darker with age. The scrotum is the most common site but lesions are also found on the penis and the vulva. Lesions can bleed but otherwise the condition is asymptomatic
• Angiokeratoma Corporis Diffusum - this is the dermatological hallmark of several rare inherited lysosomal disorders. One of the most recognised disorders is Anderson-Fabrey’s disease, in which angiokeratoma are associated with opthalmological disorders, pain (often in the digits), renal failure and severe cardiovascular events at a young age. The cutaneous eruptions usually appear before puberty and tend to occur in groups, often being described as ‘naevoid’. Occasionally cutaneous lesions may be minimal or absent

	Figure 11 – Solitary angiokeratoma
	Figure 12 - Angiokeratoma of the shin
	Figure 13 - Dermoscopic appearance of figure 12
	Figure 14 - Angiokeratoma of Fordyce
	Figure 15 – ‘Naevoid’ angiokeratoma The appearance of grouped angiokeratoma should alert the clinician to the possibility of a serious underlying disorder such as Anderson-Fabrey’s disease. All such patients warrant referral
	Figure 16 – Dermoscopic view of figure 15 showing red lacunes

Group III: Other vascular lesions – common

Diagnosis: Spider naevus (syn. spider telangiectasia)

Key diagnostic features

• A very common skin lesion
• Most often arises in children, pregnancy and liver disease
• An arcade of vessels radiates out from a central arteriole – compressing the central point will blanche the arcade
• In adults with liver disease multiple lesions develop on the upper chest and trunk
• Treatment – Most lesions do not need treating. Light electrocautery to the central arteriole and laser are effective treatments

	Figure 1 – Spider naevus on nose
	Figure 2 – Spider naevus close up Central arteriole highlighted by arrow

Diagnosis: Pyogenic granuloma

Key diagnostic features

• The lesion is a rapidly growing mass of capillaries enmeshed in connective tissue
• Most common in children, young adults and pregnancy, but can occur at any age
• Trauma is often an important initiating factor – although a clear history cannot always be ascertained
• Lesions can arise on any part of the body but the most common sites are the fingers/hands, head, neck, upper trunk and feet
• The lesion starts as a small red spot but quickly enlarges into a nodule
• The lesion is initially smooth, but often becomes eroded quickly and bleeds significantly on contact
• A small number of lesions will organise and resolve but most persist and are a nuisance needing treatment
• Although the diagnosis is often straightforward the main differential diagnosis is that of an amelanotic melanoma, which tend to bleed less than pyogenic granuloma. Other features that may increase the level of suspicion include no clear history of trauma and atypical site or age group. To this end, lesions needing treatment are best removed surgically (deep curettage & cautery or excision) and sent for histology

	Figure 3 – Early pyogenic granuloma in a 12 year old boy
	Figure 4 – Pyogenic granuloma in an elderly woman
	Figure 5 – Pyogenic granuloma A history of trauma was given

Diagnosis: Venous lake

Key diagnostic features

• Arise in older patients
• Lips, ears and face are the most common sites
• Dark-blue, soft and compressible papules

Figure 6 – Venous lake of lower lip

Group IV: Other vascular lesions – uncommon / rare

Diagnosis: Glomus tumour

Key diagnostic features

• Benign vascular lesions
• Normally present as a painful red-blue discolourisation under a nail
• X-Ray may show boney erosion
• Multiple glomus tumours can arise in children & young adults. These are larger, dark blue and non painful

Figure 1 – Glomus tumour under a nail

Diagnosis: Blue rubber bleb naevus syndrome

Key diagnostic features

• Rare
• Most commonly arises in children
• Multiple lesions – although numbers often limited
• Blue compressible nodules on any part of the body
• Lesions often quite small but occasionally can be several cm’s in diameter
• Patients also develop internal vascular proliferations, especially of the GI tract and skeletal system. Serious GI haemorrhage can occur
• Lesions remain unchanged throughout life

Figure 2 - Blue rubber bleb naevus syndrome

Diagnosis: Angiolymphoid hyperplasia (syn. epithelioid hyperplasia)

Key diagnostic features

• Rare in the UK (much more common in Japan)
• Mainly arises in young adults
• Ears and hairline most common sites
• A group of small, red-skin coloured translucent nodules
• Spontaneous regression is seen in the majority of cases

Figure 3 - Angiolymphoid Hyperplasia

Diagnosis: Angioma serpiginosum

Key diagnostic features

• Rare
• Female predominance
• Usually arises in childhood
• Multiple pinpoint red papules, which often coalesce into large sheets
• Number of lesions increase over a number of years and then remain stable

Figure 4 - Angioma Serpiginosum

Diagnosis: Lymphangioma circumscriptum

Key diagnostic features

• A localised area of lymphatic malformation
• Uncommon
• Often noted at birth but can arise in childhood
• Groups of fluid-filled vesicles bulge into the skin, which may look like frogspawn.
• Fluid can be clear-red, or blue-black if they contain blood
• Deeper involvement of the lymph system can occur, which can be associated with lymphoedema
• Although the condition is benign, SCC has been described arising from lymphangioma (very rare)
• Management is difficult. If treatment is needed radical surgery is necessary and recurrence is not uncommon

	Figure 5 – Lymphangioma circumscriptum over the abdomen
	Figure 6 – Close up of figure 5

Diagnosis: Naevus anaemicus

Key diagnostic features

• Uncommon
• Congenital
• The white appearance is caused by vasoconstriction within the naevus
• Arises most often on the trunk
• Irregular white patch with well-defined border
• The border can be obliterated by blanching the surrounding skin
• Persists into adulthood and remains unchanged. Cosmetic camouflage may be useful

Figure 7 – Naevus anaemicus of the trunk

Diagnosis: Kaposi’s sarcoma (syn. Kaposi’s haemorragic sarcoma)

Key diagnostic features

• A neoplastic process arising from vascular and lymphatic endothelium
• There are several groups:
  • The most frequent occurrence is in patients with HIV, which is associated with a poor prognosis
  • Non-HIV immunosuppression e.g. transplant patients. It can regress if immunosuppression ceases, but can be aggressive and associated with a poor prognosis
  • Sporadic – Mainly found in the elderly male Jewish population. Lesions begin insidiously around the ankle and slowly spread upwards. Lesions rarely fatal
  • Endemic- Zaire, Uganda and Rwanda. Associated with gross oedema and responds to chemotherapy.
• The extremities is the most common site / oral lesions can occur
• Dark-blue / purple colour
• Start off flat, then become raised and tumid growing up to 3cm in diameter
• Lesions tend to be multifocal and adjacent areas may fuse to form large plaques
• Lesion can become ulcerated and some involute to leave pigmented scars

	Figure 8 – Early stage Kaposi’s Sarcoma
	Figure 9 – Later Stage Kaposi’s Sarcoma
	Figure 10 – Kaposi’s Sarcoma of the mouth

Diagnosis: Angiosarcoma (syn. malignant haemangioendothelioma)

Key diagnostic features

• Very rare
• Most frequently arises in elderly men
• Head and neck most common site but can occur anywhere
• Initial presentation may be subtle with bruises / thin purple patches and plaques
• Lesions become more nodular and ulcerate with time
• Metastases are common and lesions on limbs are particularly aggressive

	Figure 11 – Angiosarcoma on the upper / outer chest (Copied with kind permission from Dermatoweb)
	Figure 11 – Angiosarcoma