Topic
Angioma serpiginosum
About: Angioma serpiginosum is a research topic. Over the lifetime, 84 publications have been published within this topic receiving 1035 citations. The topic is also known as: Angioma serpiginosum of skin & ANGIOMA SERPIGINOSUM, X-LINKED.
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TL;DR: Close similarity is apparent between the members of that group of idiopathic pigmented purpuric eruptions which includes Schamberg's progressive pigmentary disorder, angioma serpiginosum of Hutchinson, Majocchi's purpura annularis telangiectodes and pigmented Purpuric lichenoid dermatitis.
Abstract: WE HAVE been impressed with the close similarity between the members of that group of idiopathic pigmented purpuric eruptions which includes Schamberg's progressive pigmentary disorder, angioma serpiginosum of Hutchinson, Majocchi's purpura annularis telangiectodes and pigmented purpuric lichenoid dermatitis of Gougerot and Blum. The similarity is apparent both clinically (figs. 1, 2 and 3) and histologically (figs. 4, 5 and 6). It has been our experience that the presentation of any one of these conditions before a group of dermatologists usually provokes considerable debate, with infrequent agreement on the diagnosis. It is characteristic of each of these disorders that the clotting time, bleeding time, thrombocyte count and other hematologic factors are within normal limits. Acute intoxicating illness, history of drug ingestion and definite allergic manifestations are not frequent features of any one of these diseases. Except for demonstrable increased capillary permeability or fragility the etiologic factors remain obscure. These facts must
70 citations
TL;DR: The distinguishing clinical and genetic criteria of 20 different capillary malformations are described, and a specific name is given to all of them, and arguments are presented why the salmon patch cannot be categorized as a naevus.
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.
61 citations
TL;DR: A case of angioma serpiginosum involving extensive areas of the extremities and the trunk is reported to emphasize that extensive cutaneous involvement must be included in the clinical spectrum of this condition.
Abstract: Angioma serpiginosum is a rare condition that involves both the proliferation and dilatation of superficial blood vessels in the skin. It classically begins in childhood, is more common in females, and is asymptomatic. Typical lesions appear on the extremities and are often asymmetric. We report a case of angioma serpiginosum involving extensive areas of the extremities and the trunk to emphasize that extensive cutaneous involvement must be included in the clinical spectrum of this condition.
40 citations
TL;DR: This single case of cutaneous angiomata of the "port wine" variety, which may represent a new syndrome, is reported because of the unusual retinal abnormality in this patient.
Abstract: Cutaneous angiomata of the "port wine" variety are frequently associated with angiomata elsewhere in the body and particularly in the central nervous system. Retinal and nervous system involvement by angioma serpiginosum has, however, not previously been recorded. For this reason and also because of the unusual retinal abnormality in this patient, it is felt justifiable to report this single case, which may represent a new syndrome.
39 citations