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Biomedical subjects

Charles Steffen

Publications and source records attributed to Charles Steffen.

At least 19 recordsLinked to original sources

The man behind the eponym dermatology in historical perspective: Albert Sézary and the Sézary syndrome.

In a series of papers from 1938 to 1949, Albert Sézary, a French dermatologist and syphilologist, described erythroderma with cellules monstrueuses (monster cells) in the skin and blood, which is now known as Sézary syndrome or Sézary disease. This historical note reprises the life and work of Sézary. It outlines his original reports and his thoughts about the pathogenesis of the disease as a reticulosis, and lists a composite classification of the reticuloses, which includes that of Sézary. We touch briefly on the articles that first used the terms Sézary reticulosis and Sézary syndrome and the changing concepts of the reticuloses and the reticuloendothelial system. We conclude that Sézary syndrome (Sézary disease, Sézary reticulosis) cannot be separated from mycosis fungoides clinically, histopathologically, hematologically, or viscerally and, therefore, is not a disease sui generis. Despite our conclusions, present day consensus defines Sézary disease clinically as a generalized pruritic erythroderma: histopathologically with an epidermal and dermal infiltrate, lymphadenopathy and visceral involvement all containing monster cells (Lutzner/Sézary cells) in the skin, peripheral blood, lymph nodes, and viscera, a disease different and separate from mycosis fungoides.

Dermatology↗

Dermatopathology in historical perspective: epithelioma cuniculatum (Aird).

Professor Ian Aird, a renowned British surgeon of the mid-twentieth century, described a peculiar neoplasm of the foot in 3 patients under the term epithelioma cuniculatum, now more commonly called carcinoma cuniculatum. I will recount the life of Professor Aird and then summarize his original report emphasizing the clinical and more particularly the histopathologic criteria of epithelioma cuniculatum as described by Aird et al. Then I will summarize some of the subsequent reports of epithelioma (carcinoma) cuniculatum. Authors have grouped carcinoma cuniculatum with giant condyloma of the penis of Buschke-Loewenstein and oral verrucous carcinoma of Ackerman and similar neoplasms at other sites under the generic title "verrucous carcinoma." Although this classification has been accepted, I will stress that the clinical picture and histopathology described by Aird et al differ from those in later reports of carcinoma cuniculatum. These correspond to descriptions of the verrucous carcinomas of Buschke-Loewenstein and Ackerman. Finally, I will conclude that carcinoma cuniculatum as delineated by Aird and his colleagues is an extremely rare, indolent, nonmetastasizing squamous cell carcinoma composed of banal keratinocytes with unique clinical and histopathologic features that almost always arises on the foot. If a neoplasm on the foot has the characteristics of verrucous carcinoma that occurs at others sites, but not those of Aird et al, it should be called verrucous carcinoma of the foot, and not "epithelioma (carcinoma) cuniculatum."

Carcinoma, Verrucous↗

The men behind the eponym--Abraham Buschke and Ludwig Lowenstein: giant condyloma (Buschke-Loewenstein).

In 1925 Abraham Buschke and Ludwig Loewenstein described a neoplasm of the penis (carcinomähnliche condylomata acuminata des penis), which, to them, bore resemblances to common condyloma acuminatum and squamous cell carcinoma, but it had histopathologic and clinical characteristics that differed from these 2 proliferations. Later Lauren V. Ackerman described a similar neoplasm of the oral mucosa that he termed "verrucous carcinoma." I have previously reviewed that neoplasm, now referred to as "oral verrucous carcinoma of Ackerman." Still later Aird and his colleagues described a unique neoplasm of the foot, "carcinoma cuniculatum." These 3 have been grouped generically as "verrucous carcinoma." Verrucous carcinomas occur at many sites including the female genitalia and the aerodigestive system. This report will synopsize the lives of Abraham Buschke and Ludwig Loewenstein, review their original articles, and mention the changing concepts over time concerning condylomata, particularly large condylomata. Some other reports of giant condyloma (verrucous carcinoma of the penis) will be discussed, emphasizing the histopathology of this neoplasm and its differentiation from "warty-like" carcinomas of the penis. Verrucous carcinoma of other sites and epithelioma (carcinoma) cuniculatum will not be discussed.

Condylomata Acuminata↗

Ketron-Goodman disease, Woringer-Kolopp disease, and pagetoid reticulosis.

In this historical review I will synopsize the original articles by Lloyd W. Ketron and M.H. Goodman who described Ketron-Goodman disease, by Frederic Woringer and Pierre Kolopp who described Woringer-Kolopp disease, and by Otto Braun-Falco and colleagues who described pagetoid reticulosis. In their publications, each of these authors reported on one patient. I will review the clinical picture of the three patients, their histopathology, and the pathogenesis of each disease as suggested by the above authors. Then the views of others that have written on the subject recently will be reviewed particularly as to their conception of the diseases. Publications that describe the histopathology of the patch (early) stage of mycosis fungoides will be redacted to compare it to the histopathology of Ketron-Goodman disease, Woringer-Kolopp disease, and pagetoid reticulosis. I will discuss whether any or all of them are diseases sui generis, whether they are one, two, or three entities, or whether any or all are but forms of mycosis fungoides.

Epidermis↗

The man behind the eponym: Lauren V. Ackerman and verrucous carcinoma of Ackerman.

In 1948, Lauren V. Ackerman described a neoplasm of the oral mucous membrane, which he thought represented a unique type of squamous cell carcinoma and that is now termed verrucous carcinoma of Ackerman. "Verrucous carcinoma" has also been used as a generic term under which are placed giant condyloma of Buschke-Loewenstein and carcinoma cuniculatum. This report will synopsize the life of Lauren Vedder Ackerman, review the original and subsequent report by Ackerman, and discuss some other reports of oral verrucous carcinoma. Because this review is directed to histopathologists, the histopathology will be emphasized. Other aspects of the neoplasm (eg, clinical picture, incidence, etiopathogenesis, and treatment) will not be discussed, or if so, only briefly. Giant condyloma acuminatum of Buschke-Loewenstein and carcinoma cuniculatum and studies that mention the occurrence of Ackerman's verrucous carcinoma in the pharynx, larynx, and esophagus will be mentioned only in the section on classification.

Carcinoma, Verrucous↗

The man behind the eponym: C. L. Pierre Masson.

This report traces the life of Pierre Masson and mentions some of his investigations. It summarizes Masson's seminal descriptions of glomus tumor and hémangioendothéliome végétant intra-vasculaire (intravascular papillary endothelial hyperplasia), also known as Masson's pseudoangiosarcoma and the Masson lesion. Finally, we reprise Masson's view of the origins of melanocytic nevi and mention Masson's neuronevus. The literature on these subsequent to Masson's articles is not discussed.

Dermatology↗

The man behind the eponym: Lucien Marie Pautrier--Pautrier's microabscess.

Lucien-Marie Pautrier, recognized during his life as a world-renowned dermatologist and venereologist, also wrote prolifically, established and headed a leading department at the medical school of Strasbourg, built an imposing structure to house the department, traveled widely in both Europe and the United States and founded the Société des Amis de la Musique at Strasbourg. He did all this while and between the two tragic World Wars that engulfed his beloved France. In the United States, his name is eponymically associated with Pautrier's microabscesses in mycosis fungoides despite the fact that he did not first describe them.

Dermatology↗

The man behind the eponyms: Richard L Sutton: periadenitis mucosa necrotica recurrens (Sutton's ulcer) and leukoderma acquisitum centrifugum-Sutton's (halo) nevus.

Richard L. Sutton described what he termed "periadenitis mucosa necrotica recurrens," now known as major aphthosis or Sutton's ulcer in 1911. He described "leukoderma acquisitum centrifugum" (halo nevus, Sutton's nevus) in 1916. Here, we summarize the 2 publications by Sutton, trace his life story, and comment briefly on these 2 diseases.

Eponyms↗

The men behind the eponym: Francis E. Senear, Barney Usher, and the Senear-Usher syndrome.

This historical review will summarize the report of Francis Senear and Barney Usher on a disease that they called pemphigus erythematodes and which later became pemphigus erythematosus (the Senear-Usher syndrome). It will then outline the lives of these two men. Finally, it will review the literature on that condition and relate the views of various authorities as to whether pemphigus erythematosus is merely pemphigus foliaceus, a variant of pemphigus foliaceus, a syndrome combining features of lupus erythematosus and pemphigus or whether pemphigus erythematosus (Senear-Usher syndrome) is a distinct and separate disease.

Dermatology↗

The men behind the eponym: Frederick Parkes Weber and Henry a. Christian: Weber-Christian disease.

Frederick Parkes Weber described relapsing nonsuppurative panniculitis in 1925. In 1928, Henry Asbury Christian added the word "febrile" (relapsing febrile nodular nonsuppurative panniculitis), whereas Brill first coined the eponym "Weber-Christian disease" in 1936. This article traces the lives of Parkes Weber and Henry Christian and summarizes their original publications. It addresses only briefly the controversy as to whether Weber-Christian disease does or does not exist.

Dermatology↗