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Spider telangiectases

Spider angiomas (spider telangiectases) are very common in children, in pregnancy, and in patients with liver disease (Figs 15.29 and 15.30). They are usually facial and solitary or few in number in the childhood type, but may be extensive in liver disease, in which they are particularly common on the upper trunk. Any adult who develops signicant spider telangiectasias should have liver transaminases measured.

Figure 15.29 Spider telangiectases in children. (a) This spider web of vessels emanating from a central arteriolar source occurs classically on the upper cheek in children. (b) Multiple spider angiomas in a child. The dorsa of the hands are common places for spider angiomas in a child.

Figure 15.30 Spider telangiectases in an adult. The finding of multiple spider angiomas on the chest of an adult should prompt a search for liver disease.

Figure 15.31 Benign telangiectasia. (a) Facial telangiectases. (b) Partial pulsed dye laser treatment of telangiectases of the chest. Note the multiple, relatively avascular areas on this woman’s chest, each of which represented one pulse from a dye laser. Obviously, more treatments were needed.

    An arcade of vessels radiating out from a central arteriole is the characteristic appearance. Compressing the central point blanches the arcade. This radiating pattern is not seen in the other telangiectatic disorders discussed in this section. The area just below the eye on the upper cheek is a very characteristic site for spider telangiectases in children. The rest of the face, chest, and hands may be affected as well.

    No treatment is needed, and most of these lesions will fade over time. Light electrocautery with an epilating needle is quite effective. The tunable dye laser has also been used.

Benign (facial) telangiectases

Multiple telangiectasias are common on the face of fair-skinned adults. Presence on the area about the nose and cheeks is common. This is often an isolated finding, unrelated to rosacea (Fig.15.31). Solar damage is probably important.

Costal fringe

An arcade of telangiectases (Fig.15.32) on the trunk, often near the costal margin but also elsewhere on the trunk, is characteristic of this benign skin change of the elderly. The telangiectases tend to be blue rather than bright red as occurs in many other telangiectatic disorders. No treatment is needed.

Unilateral nevoid telangiectasia

Telangiectasias in this condition develop in a segmental or ‘nevoid' distribution. The onset of this condition is often associated with an elevation in hormones, for example at puberty or during pregnancy (in the latter cases, some will disappear or fade after delivery, so treatment should be avoided). Often, no treatment is necessary other than reassurance that this is a type of birthmark made apparent by the effects of estrogen. The pulsed dye laser was surprisingly ineffective in one study, with new lesions recurring soon after treatment.

Hereditary hemorrhagic telangiectasia

Etiology and pathogenesis

Patients with hereditary hemorrhagic telangiectasia (HHT), also known as Osler–Weber–Rendu disease, develop multiple cutaneous telangiectasias as well as AVMs internally. The inheritance is autosomal dominant with incomplete penetrance. Mutations in one of two chromosomal sites can cause HHT. In HHT-1, mutations at chromosome 9 alter the protein endoglin. In HHT-2, mutations at chromosome 12 alter the protein activin receptor-like kinase-1 (ALK-1). Both ALK-1 and endoglin are receptors for transforming growth factor- b (TGF- b ) and are expressed primarily in arterial endothelial cells. Endoglin and ALK-1, as well as other TGF- b signaling components, are essential during angiogenesis.

    Vascular abnormalities may affect the liver (portal hypertension), spleen, lung (pulmonary AVM and hemoptysis; Fig.15.33), gastrointestinal tract (gastrointestinal bleeding and anemia), and urinary tract (hematuria). Central nervous system abscess may occur, presumably from pulmonary AVMs that do not filter venous septic foci.

Clinical

The classic patient experiences recurrent epistaxis at a young age. Later, telangiectatic mats develop on the tongue, lips, fingertips, and elsewhere (Fig.15.34).

Figure 15.32 Costal fringe. This benign arcade of telangiectasias is often found incidentally while treating other problems.

Figure 15.33 Pulmonary arteriovenous malformations are relatively common in patients with hereditary hemorrhagic telangiectases.

Figure 15.34 Hereditary hemorrhagic telangiectasia. (a) The patient is born with a tendency to develop multiple telangiectases, usually on the face and hands. Epistaxis (nose bleeds) are extremely common in early childhood. (b) Multiple lesions on the face.

Differential diagnosis

There is very little doubt of the diagnosis for the patient who develops epistaxis in childhood, followed by the classic cutaneous telangiectasias. The main difficulty is that some children have multiple telangiectases clinically resembling HHT, usually on the dorsal forearms and hands, without epistaxis or other bleeding; it is difficult to know whether this represents a milder variation of HHT (possibly due to an as yet undescribed mutation). Adults with CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasia; Ch.13) can appear nearly identical to patients with HHT as they can have telangiectasias on the face and fingers.

Treatment

Many complications involving the brain, liver, and lung can develop. Thus a multidisciplinary approach to therapy is needed, with the primary risks being outside the dermatologist's arena. The cutaneous telangiectases may be treated with laser. DNA testing is now available, and so relatives may be screened, thus saving on radiologic imaging. Only those relatives with a positive genetic analysis need further evaluation of internal organs.

Telangiectasia macularis eruptiva perstans

This variant of mastocytosis presents with telangiectatic macules, often on the trunk, of a woman (Fig.15.35). Special stains may be needed to help identify the mast cells histologically. Clinically, one sees 5–10-mm pink-brown telangiectatic macules, predominantly on the trunk. Itching may vary from mild to intense. Gentle scratching may elicit Darier sign. The forms of mastocytosis and its investigation, differential diagnosis, and therapy are discussed in Chapter 11.

    For treatment, a potent topical steroid may be very effective. For example, clobetasol (0.05% cream applied to one section of the body every day for 1 month) can nearly clear lesions. Then another section of the body should be treated, while applying maintenance to the initial area (e.g. initially once a week down to once a month). It is important not to coat the body daily with a potent topical steroid, so as not to suppress the hypothalamic–pituitary axis. In addition, it is important to avoid prolonged daily use of such a potent topical steroid, as it may induce atrophy and telangiectasias.

Figure 15.35 Telangiectasia macularis eruptiva perstans. Fine telangiectasias coalesce to form small mats on the skin. An aggregate of mast cells in the skin is the cause

    The 585-nm flash lamp-pumped dye laser cleared all treated lesions without scarring after one treatment in one study. Doxepin given pre- and postoperatively provided the best mast cell-mediator blockade. Its effects seem to be secondary to reducing the vasculature and not via an effect on the mast cells. Any itching should be treated with a non-sedating antihistamine. PUVA may be tried. Total skin electron beam completely cleared one patient's condition.

Generalized essential telangiectasia

Telangiectases develop in the skin of patients with this benign condition. Only small areas or the entire body, including the eyes, may be involved (Fig.15.36). Inheritance patterns and clinical variants have yet to be fully defined. No systemic bleeding is usually associated, although cases have been described with similar lesions in the stomach, and one patient did develop gastrointestinal bleeding associated with a watermelon stomach.

    Hereditary hemorrhagic telangiectasia should be excluded, and a screen for liver abnormalities should be done. Individual lesions may respond to laser therapy, but treating the entire patient is usually impractical.

Figure 15.36 Generalized essential telangiectasia. (a) Innumerable telangiectases cover the legs. (b) The same patient as in (a) displaying generalized essential telangiectasia involving the eyes.

Other telangiectasias

White/Cox: Diseases of the Skin, 2ed.(c) 2006, Elsevier Inc. All rights reserved.