Degos syndrome

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Classification according to ICD-10
I77 Other diseases of the arteries and arterioles
I77.8 Other specified diseases of arteries and arterioles
ICD-10 online (WHO version 2019)

The Degos disease is a rare disease of the arterioles . Only about 130 cases of the disease have been described in the literature worldwide (as of 2009). However, there may be a far larger number of unreported cases due to undetected cases of illness. Degos syndrome is also known as "Degos-Delort-Tricot syndrome", "Papulosis atrophicans maligna", "malignant atrophic papulosis", "atrophic papulosquamous dermatitis", "Morbus Köhlmeier-Degos" and "fatal cutaneous-intestinal syndrome " known.

Synonyms are: Malignant atrophic papulosis; Degos disease; Kohlmeier-Degos disease

The disease was first described in 1940 by Walter Köhlmeier, a Viennese pathologist, as a sub-form of thrombangiitis obliterans . Shortly afterwards, the Parisian dermatologist Robert Degos recognized, regardless of Köhlmeier's descriptions, that it was an independent disease.

The disease is not to be confused with Erythrokeratoderma en cocardes Degos , a rare hereditary skin disease.

description

The occurrence of characteristic skin changes is pathognomonic . They start out as small red papules about an inch in diameter. After a few days, the center of the lesion sinks, older lesions then show a "porcelain-like" sunken center with a reddened rim wall consisting of enlarged, tiny vessels ( telangiectasia ). These Degos lesions typically affect the entire body, but save the face, soles of the feet, and palms of the hands. An infestation of internal organs can, but does not have to occur. In addition to the skin, the mucous membrane of the gastrointestinal tract and the central nervous system (CNS) can also be affected . Other organs that can be affected are the pleura , kidneys, eyes, pericardium , pancreas and urinary bladder, as is known from post-mortem examinations .

If only the skin is involved, one speaks of the benign (= benign) form, if internal organs are affected it is the malignant (= malignant) form. It remains unclear whether these are different entities or whether the benign form is in each case only a preliminary stage on the way to malignant. If internal organs are affected, death usually occurs within a few months or years. The most common causes of death are gastrointestinal perforations and cerebral insults .

Pathophysiologically , it is the thrombotic occlusion of an arteriole, which leaves a wedge-shaped area of ​​cutaneous ischemia . This is then visible as a typical Degos skin lesion. The skin lesions are neither painful nor itchy.

therapy

An effective therapy is not yet known. Numerous attempts have been made with immunosuppressants or anticoagulant drugs. However, only a platelet aggregation-inhibiting therapy with acetylsalicylic acid has prevailed . However, the results are modest here too, and due to the low number of diseases, an effective proof of effectiveness is not possible.

Individual evidence

  1. ↑ C. Viktor, U. Schultz-Ehrenburg: Papulosis maligna atrophicans (Köhlmeier-Degos). Diagnosis therapy, course . In: dermatologist. 2001; 52, pp. 734-737.
  2. ↑ papulosis, atrophic malignant. In: Orphanet (Rare Disease Database).
  3. ↑ W. Köhlmeier: Multiple skin necroses in thrombangiitis obliterans . Arch Dermatol Syph 1940; 181, pp. 783-792.
  4. ^ R. Degos, J. Delort, R. Tricot: Dermatite papulosquameuse atrophiante . In: Bulletin de la Societe francaise de dermatologie et de syphiligraphie. 1942; 49, pp. 148-150.
  5. ^ A b c E. Ball, A. Newburger, A. Ackerman, A. Bernhard: Degos' Disease: A Distinctive Pattern of Disease, Chiefly of Lupus Erythematosus, And not a Specific Disease per se. In: Am J Dermathopathol. 2003; 25 (4), pp. 308-320.
  6. ^ R. Amaravadi, T. Tran, R. Altmann, C. Scheirey: Small bowel infarcts in Degos disease. In: Abdominal Imaging. 2007.
  7. ^ T. Wachter, C. Rose, E. Bröcker, M. Leverkus: Benign course of malignant atrophic patulosis (Köhlmeier-Degos disease): Missing vascular occlusion as a favorable prognostic finding? In: JDDG. 2003; 1, pp. 374-377.

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