Capillary malformations: a classification using specific names for specific skin disorders

Abstract

The name capillary malformation has caused much confusion because it is presently used to designate numerous quite different disorders such as naevus flammeus, the salmon patch, the vascular naevus of the hereditary 'megalencephaly-capillary malformation syndrome' and the skin lesions of non-hereditary traits such as 'capillary malformation-arteriovenous malformation' and 'microcephaly-capillary malformation'. To avoid such bewilderment, the present review describes the distinguishing clinical and genetic criteria of 20 different capillary malformations, and a specific name is given to all of them. The group of capillary naevi includes naevus flammeus, port-wine naevus of the Proteus type, port-wine naevus of the CLOVES type, naevus roseus, rhodoid naevus, cutis marmorata telangiectatica congenita, congenital livedo reticularis, segmental angioma serpiginosum, naevus anaemicus, naevus vascularis mixtus and angiokeratoma circumscriptum. Capillary lesions that perhaps represent naevi are the mesotropic port-wine patch, Carter-Mirzaa macules, unilateral punctate telangiectasia and unilateral naevoid telangiectasia of the patchy type. Capillary malformations that do not represent naevi include X-linked angiokeratoma corporis diffusum (Fabry disease), autosomal dominant angiokeratoma corporis diffusum, hereditary haemorrhagic telangiectasia, hereditary angioma serpiginosusm and the salmon patch. In this way, we are able to discriminate between various non-hereditary capillary naevi such as naevus roseus and the hereditary rhodoid naevus and several hereditary traits that do not represent naevi such as angiokeratoma corporis diffusum and hereditary haemorrhagic telangiectasia; between four different types of port-wine stains, three of them being lateralized and one being mesotropic; between cutis marmorata telangiectatica congenita and congenital livedo reticularis; between telangiectatic naevi and the vasoconstrictive naevus anaemicus; and between two different types of angiokeratoma corporis diffusum. Finally, arguments are presented why the salmon patch ('stork bite', 'naevus simplex') cannot be categorized as a naevus.

Mixed vascular nevus syndrome: a report of four new cases and a literature review

“…In subsequent reports, Happle (13,14) called these lesions "vascular twin nevi". In his latest classifications of the various archetypical shapes and patterns of cutaneous nevi, Happle (2)(3)(4) categorized the telangiectatic nevus (as well as the nevus anemicus) into the configurations called "patches with indented borders" and "blocks or flags" stating that "lesions adopting these shapes may show areas of normal tissue within the patch, intermingled with involved areas, leading to a somewhat reticular pattern with preserved irregular borders" (2-4). Di Lernia et al (5) and Happle (14) further defined this skin phenotype understanding that it was characterised by a true admixture of reticular telangiectatic lesions and angiospastic spots of nevus anemicus.…”

“…Quant Imaging Med Surg 2016;6(5):515-524 qims.amegroups.com of recessive (didymotic) alleles controlling the balance between constriction (i.e., nevus anemicus) and dilatation (i.e., nevus telangiectaticus) of cutaneous blood vessels in an embryo could be the primary event causing twin spotting (i.e., paired vascular twin nevi) (Figure 1) and the appearance of the two neighbouring and partially admixed cell populations would be the secondary phenomenon (2)(3)(4)(5)10). In addition to that, similarly to what occurs in the skin, the crossed hemi-cerebral malformation (Figures 2-4) may well represent dichotomic functional abnormalities likely reflecting allelism at the same, yet undiscovered, underlying gene loci of the vascular twin nevi ( Figure 5).…”

“…These are vascular nevi of the capillary type (1)(2)(3)(4). Telangiectatic nevi are congenital skin lesions formed by small, dilated blood capillaries measuring 0.5-1 mm in diameter; these lesions may show areas of normal tissue within the affected area, intermingled with involved areas, leading to a somewhat reticular pattern with preserved irregular borders (2)(3)(4). The so-called nevus anemicus represents a vasoconstrictive counterpart of telangiectatic nevi; typically, it is a pale macule with a highly irregular margin.…”

“…The so-called nevus anemicus represents a vasoconstrictive counterpart of telangiectatic nevi; typically, it is a pale macule with a highly irregular margin. If the skin is rubbed, there is no erythematous reflex within the nevus (1)(2)(3)(4). Both nevi may occur as pure cutaneous traits (1)(2)(3)(4)(5)(6) or as a part of several genetic skin disorders (6) including phacomatosis pigmentovascularis (PPV) (e.g., phacomatosis cesioflammea and cesioanemica) (7-10) and a newly reported phenotype known as mixed vascular nevus (nevus vascularis mixtus) (1,6,(10)(11)(12).…”

The Effectiveness of Port wine Stain Laser Treatment in Children

“…Они существуют с рождения, хотя могут длительно не проявляться [3]. Наиболее распространены капиллярные мальформации, описанные в литературе как «винные пятна», «пла-менеющий невус», «капиллярные мальформа-ции» и «капиллярные ангиодисплазии» (КАД) [4]. Степень распространенности КАД -3-5 случаев на 1000 новорожденных; отличительные призна-ки -большое число аномально расширенных кро-веносных сосудов без признаков пролиферации эндотелия в папиллярном и частично ретикулярном слоях дермы, не подвергаются самопроизвольной инволюции и прогрессируют с возрастом.…”