Are You Confident of the Diagnosis?

What you should be alert for in the history

Widespread symmetrical cutaneous telangiectasias can be seen in plaque-like, macular, retiform, annular, sheet-like, or net-like patterns. They are usually asymptomatic; however, some individual lesions can cause tingling, burning, or numbness.

Characteristic findings on physical examination

Characteristic findings include widespread cutaneous telangiactasias that are pink or red in color and easily blanched with rapid refilling (Figure 1).

Figure 1.

Generalized essential telangiectasia. (Courtesy of Bryan Anderson, MD)

Telangiactasias favor lower extremities and are symmetrical. They are usually permanent and can progress, become more prominent with dependent positioning, and occur on mucous membranes.

There is an absence of concurrent epidermal and dermal changes, e.g. purpura, atrophy, depigmentation, or follicular involvement, and an absence of systemic symptoms or concurrent diseases.

Expected results of diagnostic studies

Laboratory studies, including coagulation, are usually unremarkable.

Histopathology shows telangiectatic vessels in the reticular and papillary dermis; as well as a paucity of inflammation.

Diagnosis confirmation

Differential Diagnosis:

• Angiokeratoma corporis diffusum (Fabry syndrome) - presents early in life (inborn error of metabolism, X-linked), lesions are hyperkeratotic with lack of blanching

• Cutaneous collagenous vasculopathy - may be clinically identical; however, collagen is appreciated around the postcapillary venules histologically

Who is at Risk for Developing this Disease?

There is a wide age distribution, with a median age of onset of 38 years.

Telangiectasias have a female predominance and a Caucasian predominance. Familial cases with an autosomal dominant inheritance pattern have been described

What is the Cause of the Disease?

Etiology

The etiology is unknown. A role for sun exposure has been suggested.

Pathophysiology

The pathophysiology includes widespread dilation of dermal vessels.

Systemic Implications and Complications

Telangiectasias are usually asymptomatic; there was one case report of associated gastrointestinal (GI) bleeding.

Treatment Options

Treatment is usually unnecessary. The lesions are often refractory to hemoglobin-targeting laser therapy, especially those localized to the lower extremities; moreover, given the widespread distribution, this is usually impractical.

Sclerotherapy is contraindicated due to the risk of worsening exisiting lestions and/or creating new ones.

Optimal Therapeutic Approach for this Disease

Reassurance is key. Monitor for possibility of internal bleeding, although this is an exceedingly rare complication.

Treatment is usually unnecessary; however, hemoglobin-targeting laser therapy can be considered, especially for facial lesions. Sclerotherapy is contraindicated.

Patient Management

Reassurance is key. Treatment is usually unnecessary. There is a low risk for internal bleeding.

Unusual Clinical Scenarios to Consider in Patient Management

There was one case report of associated GI bleeding. Systemic complications are very rare.