Ann Dermatol Venereol 1988 ;115(9):909-17

Service de Dermatologie, Faculté de Médecine, Cliniques universitaires, Bruxelles.

Six patients of the same family present with micronodular and generalized familial angiomatosis. Four of them have been investigated. Their problem is purely esthetic; however an asymptomatic form of von Willebrand disease has been found in a female and one of her daughters. The light microscope reveals a network of dilated capillaries in the superficial dermis. Electron microscope investigation of the endothelium demonstrates on abundance of Weibel-Palade bodies, the presence of osmiophilic inclusions within clear vacuoles, the occurrence of long spacing collagen fibrils in the vicinity of endothelial and perithelial cells; furthermore, cytoplasmic projections within the lumen constitute the most dramatic and constant feature: there are many villosities, loops, coils, tufts and entanglements in all four cases.
March 1989
2 Reads

Similar Publications

Ultrastructural study of hemangiomas. 1. Capillary hemangioma of the skin.

Acta Pathol Jpn 1981 Jan;31(1):105-15

The fine structure of capillary hemangiomas from the skin of six young adults was investigated. All the vessels had non-fenestrated endothelial cells. Their intercellular junctions had tight junctions, but desmosome-like structures were also present. Read More

View Article
January 1981

Fibrous long-spacing collagen in bacillary angiomatosis.

Ultrastruct Pathol 1998 Mar-Apr;22(2):127-33

Albert Einstein College of Medicine, Bronx, New York, USA.

Fibrous long-spacing (FLS) collagen is a distinct ultrastructural form of collagen present in normal tissue, various tumors, and tissues degraded by bacterial collagenases in vivo and in vitro. An association between FLS collagen and bacillary angiomatosis has not been previously described. Six cases of bacillary angiomatosis, including one autopsy case with disseminated disease, were examined ultrastructurally. Read More

View Article
August 1998

[Sarcomatous degeneration of a familial cavernous angiomatosis (Bean's syndrome) (author's transl)].

Authors:
L Sarrat P Sarrat

Ann Dermatol Venereol 1980 Apr;107(4):259-65

Although the classification of "Ullman's universal angiomatosis" has not yet been completely defined, the authors estimed it interesting to report an observation which may fall into this denomination. This observation concerns a generalized cavernous angiomatosis differing from hereditary hemorrhagic telangiectasia (Rendu-Osler type) with its conventional skin and mucous membrane manifestations and its visceral forms. The patient presented cutaneous and probably digestive generalized cavernomatosis related to blue rubber bleb naevus. Read More

View Article
April 1980

Immunolocalization of von Willebrand protein in Weibel-Palade bodies of human endothelial cells.

J Cell Biol 1982 Oct;95(1):355-60

Immunofluorescence staining of cultured human umbilical vein endothelial cells has shown the presence of von Willebrand protein in the perinuclear region, in small rodlike structures through the cytoplasm, and on filaments of the extracellular matrix. Nonendothelial cells showed no staining with anti-von Willebrand protein antiserum. At the light microscope level, immunoperoxidase treatment of endothelial cells revealed the same pattern and antibody specificity as the fluorescence staining. Read More

View Article
October 1982