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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
1929: atrophie blanche
The term "atrophie blanche" was first used by Milian, a French dermatologist, in 1929. [
] He had observed that white atrophic lesions, ranging from the size of a pinhead to that of a lentil, may be encountered in a variety of conditions, among them varicose veins and what he called "disorders of circulation." Those lesions seemed to appear most commonly on the legs but sometimes he also had seen them on the face. Milian divided atrophie blanche into two types, namely, a localized variant that he termed "atrophie blanche
and a more extensive manifestation that he called "atrophie blanche
in which an entire foot or a third of the leg (i.e., a "segment") was caught up in the process.
Milian emphasized that the white lesions observed by him were not consequent to a wound but developed without previous injury from the outside. From Milian's original description, it is difficult to understand precisely what he observed, and no pictorial material accompanied the original article. Even though Milian grouped atrophie blanche among what he called "
" (a term he defined as "inflammation of lymphatics, arteriols, or venules"), he was not certain about the pathogenesis of the condition. He took note of "sclerosis of vessel walls" and of infiltrates of inflammatory cells around vessels but, nevertheless, he thought that the disease might be related to syphilis.
In subsequent years, the term atrophie blanche came to be used primarily in the French literature of dermatology
(see also capillarite télangiectasique et atrophiante
capillarites sclérosantes et atrophiantes).
Nödl, in 1951 in an article written in German, was the first non-French colleague who employed the term atrophie blanche to designate a feverish condition in a young woman who developed scarring plaques on legs and feet. [
] What he pictured, however, is very different from what was described by Milian and other French colleagues under the diagnosis of atrophie blanche, and a diagnosis cannot be made with surety in retrospect. In 1952, Wilson presented to the Philadelphia Dermatologic Society a patient whom he had diagnosed with atrophie blanche, the first report of atrophie blanche in the United States. His article, however, was brief and did not include any pictorial material. [
] In the English literature of dermatology, the first extensive article about atrophie blanche was written by Nelson, in 1955. [
] He told of six patients, all of whom had white scars on the legs. Some of the patients also had varicose veins, while others presented with purpuric patches and hemorrhagic blisters and bullae. From the illustrations included in this article, a reader cannot be certain that all patients really had the same disease, but at least two of them had presented themselves in a very similar fashion, to wit, hemorrhagic lesions including blisters preceded the appearance of white atrophic macules. That description by Nelson is very similar to what later was designated livedo vasculitis
livedo vasculitis). Nelson noted that atrophie blanche is "more a clinical than a pathologic entity," and he considered the findings histopathologic to be not diagnostic. Proppe und Nückel, in 1956, wrote in German about "sclerosis of the dermis" in patients with varicose veins and, in their article, they also made reference to "atrophie blanche of Milian." [
] They thought that Milian had essentially observed the same condition that they did. Comparing the observations by Proppe and Nückel with those of Nelson, it is obvious that the authors were referring to two different conditions, both of which presented with white scars but only one of which was associated with varicosis, while the other was typified by hemorrhagic papules, vesicles and, at times, bullae, before white atrophic patches appeared.
More recently, atrophie blanche has been used in a variety of ways: as a descriptive term for lesions clinical in patients with and without varicosis, as a title for a distinctive clinicopathologic entity, which some authors believed to be the same disease as livedo vasculitis whereas others attempted to set forth criteria for differentiation of atrophie blanche from livedo vasculitis
livedo vasculitis). In German-speaking dermatology, the term atrophie blanche is often referred to as part of the signs clinical of long-standing varicosis along with "
purpura jeune d'ocre,"
"hypodermitis sclerodermiformis," and ulceration. Contrary to that, in English-speaking dermatology, atrophie blanche is commonly used synonymously with livedo vasculitis. That discordance in terminology may be explained by imprecise definitions and by the lack of sufficient pictorial material in the very early descriptions mentioned above. There is still no agreement about the definition and usage of the term atrophie blanche. Nevertheless, it continues to be used.
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