Angiokeratosis Naeviformis – A Form of Naevus Telangiectaticus Lateralis (Naevus Flammeus)

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Abstract
Angiokeratoma corporis neviforme was first described by Fabry in 1915. Later a variety of lesions ranging from small blood-colored spots to large tumor-like cornified lesions was described. Distribution was unilateral and often characterized by the linear arrangements of the lesions. The diagnoses chosen varied with the concept of the basic pathology. In some instances the larger lesions were considered true angiomas. Four new cases are described and the literature reviewed. Evidence of concurrent dysplasias and varicose veins was revealed as in syndromes with nevi flammei, i.e. the syndromes of Klippel-Trenaunay and Parkes-Weber. The additional neurological disorder may perhaps establish a relationship with the condition of Sturge-Weber. In one of the new cases nevi flammei, unilateral angiokeratomas and dysplasias with epilepsy were found in the same patient. The angio-keratotic skin changes are therefore considered to be a special form of nevus flammeus. Angiokeratosis neviforme is proposed as a more suitable name. This condition, as also the nevus flammeus, must be recognized as a skin manifestation of larger, usually ipsi-lateral developmental deviations of vessels and related mesenchymal tissues.

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