Vascular Dermatology

Skin Manifestations
of Venous Disease

Patients with venous disease often display dermatological changes, which can sometimes be the only indication of an underlying condition. Venous insufficiency is the most common venous disease presenting with skin changes, including venous eczema, lipodermatosclerosis and venous ulcers. Skin changes are among the earliest signs of venous hypertension. While some are easily identified, others such as acroangiodermatitis and pigmented purpuric dermatoses are less common and more difficult to diagnose. Dilation of venous structures is not always caused by venous incompetence and may be primary, or secondary to systemic disease or environmental factors such as actinic damage and radiation. Correct identification of these skin changes is essential in reaching the right diagnosis and forming an appropriate management plan.

Capillarity’s and Pigmented Purpuric

Dermatoses

Pigmented purpuric dermatoses (PPD) are a group of conditions characterised by the leakage of red blood cells and marked haemosiderin pigmentation of the skin, also referred to as capillaritis, although there is no evidence of a true vasculitis. The hallmark of PPD is its distinctive orange-brown, speckled, cayenne pepper-like discolouration. The exact cause remains unknown, however contributing factors may include venous hypertension, exercise and lower limb dependency. Medications such as Aspirin, Glipizide, Thiamine and Interferon-alfa are believed to be associated with the initiation of PPD, while conditions including diabetes mellitus, rheumatoid arthritis, hepatic disease and hyperlipidemia are also considered to have a correlation.

Mottled Skin

Cutaneous patterns are often the only indicator of a complex underlying vascular condition. A reticulate pattern is one of the most important dermatological signs of venous or arterial pathology involving the cutaneous microvasculature, and may be the sole sign of a significant underlying condition.

A range of conditions can present with a reticulate eruption, from vascular malformations such as cutis marmorata congenita telangiectasia and benign forms of livedo reticularis, to more serious conditions such as Sneddon’s syndrome.

Other Vascular Skin Conditions

Leg Ulcers

Swollen Limb

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Capillarity’s and Pigmented Purpuric

Pigmented purpuric dermatoses (PPD) are a group of conditions characterised by the leakage of red blood cells and marked haemosiderin pigmentation of the skin, also referred to as capillaritis, although there is no evidence of a true vasculitis. The hallmark of PPD is its distinctive orange-brown, speckled, cayenne pepper-like discolouration. The exact cause remains unknown, however contributing factors may include venous hypertension, exercise and lower limb dependency. Medications such as Aspirin, Glipizide, Thiamine and Interferon-alfa are believed to be associated with the initiation of PPD, while conditions including diabetes mellitus, rheumatoid arthritis, hepatic disease and hyperlipidemia are also considered to have a correlation.

Several clinical subtypes are recognised, all sharing similar histological features including lymphocytic inflammation, red blood cell extravasation and haemosiderin deposition:

Schamberg’s Disease may occur at any age but is more common in males around 40 years of age. It presents as irregular orange-brown patches predominantly on the lower limbs, spreading slowly. Lesions are chronic and may persist for years, though some may spontaneously clear. Associations include venous disease, food additive hypersensitivity and occult infections.

Itching Purpura of Lowenthal presents with more widespread red-brown lesions and severe itching. It is more common in middle-aged males around 50 years of age, with associations including clothing dermatitis and contact dermatitis to rubber.

Lichen Aureus typically occurs in patients in their 20s to 30s, presenting as a solitary golden plaque or localised group of lesions on the lower legs, often associated with an underlying incompetent perforator vein. Colour may vary from yellow to purple and some patients report pain. Treating the underlying perforator incompetence can help resolve the lesion.

Majocchi Disease commonly affects children, adolescents and young adult females around 30 years of age. It presents as small annular plaques of purpura with prominent telangiectasia, most often found symmetrically on the lower extremities, though it may extend to the trunk and upper body.

Pigmented Purpuric Lichenoid Dermatosis of Gougerot and Blum primarily affects middle-aged men around 40 years of age and has not been reported in children. It presents as thickened, itchy, eczema-like lichenoid purpuric papules on the lower legs. Contact dermatitis to oils and dyes are thought to play a role in its development.

Eczematoid like Purpura of Doucas and Kapetanakis is most common in adult females around 40 years of age and is more generalised. It is distinguished from Schamberg’s disease by the presence of focal spongiosis and scale crust.

Linear Dermatoses is a rare form of PPD presenting as asymptomatic lesions on the lower limb and torso. It generally affects young patients and typically resolves spontaneously within three months.