Cutaneous extravascular necrotizing granuloma (Winkelmann granuloma): confirmation of the association with systemic disease.

BACKGROUND: An unusual palisading granuloma has been described in patients with immunoreactive diseases. Multiple names have been given to this lesion. OBJECTIVE: Our aim was to verify whether a distinct palisading granuloma can be used as a marker for systemic disease. We also propose unifying nomenclature. METHODS: Thirty-four biopsy specimens from 22 patients were selected for study on the basis of histologic criteria. The medical histories of these patients were subsequently reviewed for clinical information. RESULTS: At least 21 of the 22 patients with cutaneous extravascular necrotizing granuloma had evidence of an underlying immunoreactive systemic illness. In each, the systemic disease preceded or was diagnosed concurrently with the cutaneous lesions. CONCLUSION: The cutaneous extravascular necrotizing granuloma has unique clinical and histologic features. In a great majority of cases, a systemic immunoreactive disease is present.

Sneddon syndrome.

Sneddon syndrome--cerebrovascular lesions and livedo racemosa--is a distinctive and uncommon disorder delineated by Sneddon in 1965. The clinical hallmarks are generalized livedo racemosa and central nervous system ischemia. Cutaneous vascular changes begin with intimal endothelial proliferation and fibromucinous matrix formation, leading to obstruction and obliteration of the vessel. The disorder is slowly progressive. No effective treatment is available, but platelet-inhibiting agents or newer antithrombotic agents may offer some hope in preventing or minimizing serious sequelae of this disease.

Pigments of Staphylococcus aureus, a series of triterpenoid carotenoids.

The pigments of Staphylococcus aureus were isolated and purified, and their chemical structures were determined. All of the 17 compounds identified were triterpenoid carotenoids possessing a C30 chain instead of the C40 carotenoid structure found in most other organisms. The main pigment, staphyloxanthin, was shown to be alpha-D-glucopyranosyl 1-O-(4,4'-diaponeurosporen-4-oate) 6-O-(12-methyltetradecanoate), in which glucose is esterified with both a triterpenoid carotenoid carboxylic acid and a C15 fatty acid. It is accompanied by isomers containing other hexoses and homologs containing C17 fatty acids. The carotenes 4,4'-diapophytoene, 4,4'-diapophytofluene, 4-4'-diapophytofluene, 4-4'-diapo-zeta-carotene, 4,4'-diapo-7,8,11,12-tetrahydrolycopene, and 4,4'-diaponeurosporene and the xanthophylls 4,4'-diaponeurosporenal, 4,4'-diaponeurosporenoic acid, and glucosyl diaponeurosporenoate were also identified, together with some of their isomers or breakdown products. The symmetrical 4,4'-diapo- structure was adopted for these triterpenoid carotenoids, but an alternative unsymmetrical 8'-apo-structure could not be excluded.

Proposed pathway of triterpenoid carotenoid biosynthesis in Staphylococcus aureus: evidence from a study of mutants.

Mutants of Staphylococcus aureus were isolated which showed changes in pigment composition compared with the parent strain. On the basis of differences in their triterpenoid carotenoid composition they were classified into seven types. In five of these types, there appeared to be a blockage in the biosynthetic pathway which resulted in the absence of some products and accumulation of others. The changes in the other two types appeared to be a consequence of some change in regulation. A scheme for the biosynthesis of triterpenoid carotenoids is presented in which the first C30 intermediate, 4,4'-diapophytoene, is converted via 4,4'-diapophytofluene, 4,4'-diapo-zeta-carotene, 4,4'-diaponeurosporene, 4,4'-diaponeurosporenal, 4,4'-diaponeurosporenoic acid, and glucosyl-diaponeurosporenoate to the major pigment staphyloxanthin.