Six cases of malignant atrophic papulosis (Degos'disease) occurring in one family

Kisch, L.S.; Bruynzeel, Derk P.

doi:10.1111/j.1365-2133.1984.tb06611.x

Cited by 43 publications

(25 citation statements)

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“…An der Spitze des Kegels im tiefen Korium lässt sich auch in der Übersicht ein teils rekanalisiertes Gefäß (Doppelpfeil) erkennen, welches von einer lymphozytären Entzündungs-reaktion umgeben ist dingbare Verlaufskontrollen darstellt! Beschränken sich die Läsionen, wie bei diesem mehr als 5 Jahre beobachteten Patienten, lediglich auf die Haut, so ist diese Verlaufsform der Erkrankung eher als "benigne" einzustufen, entsprechend einiger anderer Fallberichte mit ausschließlicher Hautmanifestation ohne letalen Verlauf [1,13,14,19,24,25,26].…”

Section: Nomenklaturunclassified

Papulosis maligna atrophicans Degos

Mensing

2002

Der Hautarzt

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Section: Nomenklaturunclassified

Papulosis maligna atrophicans Degos

Mensing

2002

Der Hautarzt

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“…The first manifestation of MAP usually occurs between the 20th and 50th year of life [3,4], while single cases with MAP in newborns and children have also been described [5,6]. A genetic predisposition with an autosomal dominant trait has been suggested, since there has been reports about more frequently affected 1st degree relatives [7-9]. …”

Section: Introductionmentioning

confidence: 99%

Malignant atrophic papulosis (Köhlmeier-Degos disease) - A review

Theodoridis

Makrantonaki

Zouboulis

2013

Orphanet J Rare Dis

172

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Definition of the diseaseMalignant atrophic papulosis (MAP), described independently by Köhlmeier and Degos et al., is a rare, chronic, thrombo-obliterative vasculopathy characterized by papular skin lesions with central porcelain-white atrophy and surrounding teleangiectatic rim.EpidemiologyLess than 200 cases have been described in the literature. The first manifestation of MAP usually occurs between the 20th and 50th year of life.Clinical descriptionThe cutaneous clinical picture is almost pathognomonic. The histology is not consistent but in most cases it shows a wedge-shaped connective tissue necrosis in the deep corium due to a thrombotic occlusion of the small arteries. In the systemic variant, manifestations mostly occur at the intestine and central nervous system.EtiologyThe etiopathogenesis of the disease remains unknown, a genetic predisposition may occur. Vasculitis, coagulopathy or primary dysfunction of the endothelial cells have been implicated.Diagnostic methodsDiagnosis is only based on the characteristic skin lesions.Differrential diagnosisIt depends on the clinical presentation of MAP, but systemic lupus erythematosus and other connective tissue diseases need to be considered.ManagementNo effective treatment exists for the systemic manifestations, while compounds that facilitate blood perfusion have achieved a partial regression of the skin lesions in single cases.PrognosisAn apparently idiopathic, monosymptomatic, cutaneous, benign variant and a progressive, visceral one with approx. 50% lethality within 2–3 years have been reported. Systemic manifestations can develop years after the occurrence of skin lesions leading to bowel perforation and peritonitis, thrombosis of the cerebral arteries or massive intracerebral hemorrhage, meningitis, encephalitis, radiculopathy, myelitis.

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“…Small-and mediumsized vessels are occluded [23,24], but the cause is unknown [23,25]. A few familial cases have been reported [26,27].…”

Section: Systemic Diseasementioning

confidence: 99%

Dermatologic Problems in the Intensive Care Unit: Part II

Berman

Silvestri

1986

J Intensive Care Med

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Skin disorders are present in many patients hospitalized in the intensive care unit. They range in severity from being the reason for admission to being a nuisance during care. These cutaneous problems have been categorized into four groups: (1) serious skin diseases that may incur life-threatening complications; (2) subtle skin problems associated with systemic disorders that may be characterized by critical events; (3) prominent cutaneous manifestations that accompany lifethreatening systemic diseases; and (4) skin disorders that develop as complications during intensive care. Diseases in the first category were discussed in Part I of this article [1]. In Part II, we discuss diseases in the second category. The remaining disorders will be covered in Parts III and IV.

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Six cases of malignant atrophic papulosis (Degos'disease) occurring in one family

Abstract: A family is described in which a mother and five of her children were affected by malignant atrophic papulosis. This suggests that genetic factors might be involved in this condition.

Cited by 43 publications

References 6 publications

Papulosis maligna atrophicans Degos

Papulosis maligna atrophicans Degos

Malignant atrophic papulosis (Köhlmeier-Degos disease) - A review

Dermatologic Problems in the Intensive Care Unit: Part II

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