Unilateral naevoid telangiectasia syndrome I78.8

Mazereeuw-Hautier 2003

Extensive, unilateral, either congenital or acquired districts with dense telangiectasias.

Unknown, often estrogen-induced manifestation. A genetically fixed, increased number of estrogen or progesterone receptors in the affected vessels is discussed.

Occurs mainly during pregnancy, puberty and alcohol-related liver diseases. Rarely present congenitally.

Preferably face, neck, shoulder-arm region, but also lower extremity and trunk. The arrangement of the lesions largely follows the dermatomes (trigeminal region,_C3,_C4, thoracic dermatomes, etc.)

Discrete or pronounced, usually sharply defined erythema, composed of fine and very fine, often star-shaped branched telangiectasias, which largely disappear under glass spatula pressure. No involvement of internal organs or the CNS. No endocrinopathies in congenital forms. Quadrant and hemiplegic formations as well as appearances along the Blaschko lines are possible.

Nevus flammeus; Bloom syndrome; Ataxia teleangiectatica; Teleangiectasia hereditaria haemorrhagica; Nevus anaemicus; Nevus teleangiectaticus; essential teleangiectasia; Angioma serpiginosum; Teleangiectasia macularis eruptiva perstans.

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