Degos, robert

(¤ 1904, † 1987) Dermatologist, active in Paris. Born in Béarn/Pyrenees.

Robert Degos was one of the great personalities of European dermatology. He began working at L'Hôpital Saint Louis near Milan in 1931 and obtained his doctorate in 1933. The subject of his doctoral thesis was "Les erythrodermies primitives streptococciques". It was inspired by his teacher at the time, G. Milian. In 1933 he moved to Henri Gougerot. From 1936 he headed the department at the Hôpital Saint Louis that had previously been headed by Arnault Tzanck, who was forced into exile in Chile for political reasons. Appointed director of the department in 1951, succeeding his teacher Gougerot.

Clinical observations: With J. Delort as assistant and R. Tricot as resident, R. Degos presented to the Société Française de Dermatologie a 49-year-old man with a skin rash of a few months old and relapsing: "We present to the Society a patient suffering from a skin rash that does not seem to fit into any known dermatological framework. It appears to be an as yet undescribed dermatosis. The proposed name is "atrophic papulo-squamous dermatitis". The histologic examination led him to conclude that "the lesions were absolutely unique". Seven months after the first skin symptoms, an acute intestinal disease occurred, leading to a laparotomy, which revealed numerous small necroses in the small intestine. The patient died six days later. This fatal course was described by R. Degos on May 21, 1942 under the heading "atrophic papulosquamous dermatitis".

On January 15, 1948, A. Tzanck, A. Civatte and E. Sidi presented the clinical picture of a 24-year-old man to the Société Française de Dermatologie with identical features to those observed by Degos. Three weeks later, however, the patient developed an acute abdomen and died within a few days.

In 1948, R. Degos, J. Delort and R. Tricot presented a paper on the same subject to the Société Médicale des Hôpitaux de Paris entitled "papulose atrophiante maligne (syndrome cutanéo-intestinal mortel)". In an article published in the "Annales de Dermatologie" in 1952, they again used this name, which was then generally accepted. Later, the name "Degos disease" or "Degos's disease or syndrome" became established in the Anglo-Saxon literature. Today, the disease is referred to as Köhlmeier-Degos disease.

It is worth mentioning that Walter Köhlmeier, a Viennese pathologist, published an article entitled"Multiple skin necroses in thromboangiitis obliterans" in the Archiv für Dermatologie und Syphilis as early as 1941. It was about a 22-year-old man with skin lesions who died after one year of intestinal perforations with identical histologic lesions on the skin and in the small intestine. In the discussion, the author rules out periarteritis nodosa. Köhlmeier was a pathologist (his work originates from the Institute of Pathology at the University of Vienna under the direction of Prof. Chiari) and did not relate the skin symptoms to the overall clinical picture. Originally, however, the patient was admitted to the Department of Dermatology of the Vienna City Hospital (Prof. Matras).

In his book Dermatology in General Medicine, T.B. Fitzpatrick speaks of the "syndrome described by Köhlmeier in 1941 and recognized as a specific entity by Degos in 1942".

Another disease of Degos, which is also listed in Rook's treatise as "Degos's syndrome", is the one that he described together with O. Delzant and H. Morival on December 11, 1947, to the Société de l'École de l'Esteban. December 11, 1947 to the Société Française de Dermatologie as "errythème desquamatif en plaques congénital et familial (génodermatose nouvelle?)" with the following comment: "la génodermatose dont nous faisons ici la description ne semble ne pas avoir été encore isolése. It is characterized by large, round, erythematous plaques, the center of which is covered by a broad scale giving some elements a cockade-like appearance". The authors distinguish this disease from Mendes da Costa's erythrokeratodermia variabilis.

Less well known is "Dowling-Degos disease", which was so named in 1974 by E. Wilson Jones. He took up the description published in 1954 by R. Degos and B. Ossipovski in the Annals of Dermatology as "reticular pigmentary dermatosis of the folds which differs from acanthosis nigricans only by a slight papillary hypertrophy and the coexistence of atrophy". G.B. Dowling had previously seen three patients, two of whom suffered from pigmentation of the axillary and anogenitocrural folds, and published his observations together with Walter Freudenthal in the British Journal of Dermatology in 1938, clearly distinguishing this disease from acanthosis nigricans.

In 1973, Pinol Aguade and Fernandez published the case of a slightly different patient in "Medicina Cutanea" under the title "Enfermedad de los puntos pardos: Dark Dot's Disease". In July 1974, E. Wilson Jones and K. Grice presented a paper at the British-French Dermatology Days in London, which was later published in the British Journal of Dermatology under the title "Reticulate Pigmented Anomaly of the Flexures (Dowling-Degos): a new genodermatosis", much to the astonishment of R. Degos. Both authors used the same title for their article published in 1978 in the Archives of Dermatology, in which they proposed the acronym "D.D.D." for both Dowling-Degos Disease and Dark Dot Disease.

The name Degos can still be found in many publications on cutaneous mastocytoses, to whose classification he contributed. In particular, Degos separated urticaria pigmentosa from tumorous mastocytoses such as: mastocytomas, papular and nodular mastocytoses and especially from diffuse cutaneous mastocytoses, for which he had proposed the term "mast cell reticulosis" on July 5, 1951, together with E. Lortat-Jacob and Mallarmé, which is of course rejected today.

The name of R. Degos could also be linked to a particular form of elastoma, the so-called elastoma of the nose, which was the subject of several publications bearing the joint signatures of J. Delort, J. Civatte and S. Bélaïch, both before the Société Française de Dermatologie in 1966 and 1970 and in a meeting in honor of Jean Pérard, the reports of which appeared in the Archives Belges de Dermatologie in 1970.

R. Degos was also the first to describe another syndrome in 1957, the concept of which he had developed together with E. Lortat-Jacob and J. Delort: the so-called Morbihan syndrome. This was an unofficial name for internal use by the department, applied to several patients from the same region who presented with edematous erythema of the upper face of unknown etiology.

This list is of course not exhaustive, but is only intended to give an idea of R. Degos' considerable contribution to dermatology, the importance of which is not disputed by anyone.

The following diseases rightly bear the name of R. Degos:

• Erythème desquamative en plaque congénital et familial,
• Papulosis maligna atrophicans (Köhlmeier-Degos disease)
• M. Dowling-Degos.

1. Civatte, J (2020) Quelques aspects de la contribution de Robert Degos à la Dermatologie. https://www.biusante.parisdescartes.fr/sfhd/ecrits/quelques-aspects-de-la-contribution-de-robert-degos-a-la-dermatologie .