< Current issue
Dermatopathology: Practical & Conceptual July - September 2005
4. Understanding livedo vasculitis: Part I—A glossary, in historical perspective, of terms related to "livedo" and "livedo vasculitis"
K. C. Nischal, M.D.
Almut Böer, M.D.
1860: livedo reticularis
1907: livedo racemosa
1929: atrophie blanche
capillarite télangiectasique et atrophiante
capillarites sclérosantes et atrophiantes
1953: capillaritis alba
1955: livedo reticularis with summer ulceration
1956: livedo reticularis with ulcerations
1957: atrophia alba
1965: Sneddon syndrome
1966: periodic painful ulcers of lower extremities
1967: livedo vasculitis
1967: segmental hyalinizing vasculitis
1974: livedoid vasculitis
1974: livedo reticulosis
1974: vasculitis of atrophie blanche
1983: PURPLE (painful purpuric ulcers with reticular patterning on the lower extremities)
1992: livedo vasculopathy
1998: livedoid vasculopathy
Suggestion for terminology
I. Useful terms
II. Confusing terms—to be avoided
III. Antiquated terms—not to be used any more
1965: Sneddon syndrome
In 1965, Sneddon told of his experience with 6 patients all of whom had presented themselves with what he called "cerebro-vascular lesions and livedo reticularis." [
] His patients had neurologic signs and symptoms associated with a widespread branched pattern of livedo, which correctly should have been termed livedo racemosa, not livedo reticularis
livedo racemosa) (
). None of the patients presented with ulceration of the skin in zones of livedo racemosa. A similar combination of signs and symptoms had already been told of by Champion and Rook, in 1960. [
] Sneddon as well as Champion and Rook, suggested that the condition may be the result of an arteriolitis, even though they noted similarities of features clinical to periarteritis nodosa. Zelger et al., in 1992, demonstrated that small to medium-sized arteries situated at the dermis-subcutis border are involved in Sneddon syndrome, and not arterioles or venules. [
] The same authors acknowledged, in 1993, [
] that in the same patient, features of Sneddon syndrome and livedo vasculitis may appear together, even though they were convinced that livedo vasculitis affected venules mostly and was not regarded by them to be a vasculitis. Whether or not there is a relationship of Sneddon syndrome to livedo vasculitis is not yet understood, but it is well known that both conditions may be accompanied by livedo racemosa, and both conditions have been encountered in patients with lupus erythematosus. The relationship between Sneddon syndrome and periarteritis nodosa is equally opaque. It has been suggested that Sneddon syndrome may be caused by a variety of factors such as coagulopathies, atherosclerosis, or embolization, e.g., in an arterial myxoma. [
] In sum, in the contemporary literature of dermatology and dermatopathology, the term Sneddon syndrome is used as a description clinical for the presence together of livedo racemosa with neurologic signs and symptoms. Whether the "syndrome" represents a distinctive disease entity or rather a pattern produced by a variety of etiologic agents, is not yet understood.
The pattern of livedo shown by Sneddon in his article of 1965 is ramified, not netlike. The correct designation of it is livedo racemose, not livedo reticularis. The patients reported on by Sneddon had neurologic signs and symptoms, but no ulcerations on the ankles so typical of livedo vasculitis. Note, however, that the lesions of livedo racemosa seen on the buttocks of this patient are very similar to those shown in Figure 3 B on the legs of one of the patients studied by Feldaker et al.
This site is made possible in part by:
Copyright © Derm101.com. All Rights Reserved.