Familial malignant atrophic papulosis (Degos' disease)—additional evidence for heredity and a benign course

Is Degos' disease a clinical and histological end point rather than a specific disease?

“…3,4 Such cases have often demonstrated a strong hereditary component. 5,6 Investigators have estimated that approximately 4-15% of patients with lesions consistent with Degos' disease enjoy continued relative health. 3,4,21,26 While it is impossible to predict with certainty which patients will progress to fulminate disease, evidence suggests a lack of visceral involvement two years after diagnosis portends a better prognosis.…”

“…Many Degos' disease variants have been detailed, including benign cutaneous Degos' disease, familial Degos' disease, atrophie blanche with Degos-like features, systemic lupus erythematosus with Degoslike lesions, dermatomyositis with Degos-like lesions, and other connective tissue diseases with similar findings. [3][4][5][6][7][8][9][10][11][12] The course, prognosis, and treatment in these cases has substantially varied.…”

Papulosis maligna atrophicans Degos

“…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].…”

Degos’ disease (malignant atrophic papulosis) as a fatal cause of acute abdomen: Report of a case

“…4,5 According to our review of the literature, most cases have developed sporadically, but there are also reports of familial Degos' disease. [4][5][6][7][8][9] Age at diagnosis ranges from 3 weeks to 67 years, which includes sporadic reports of infants and patients older than 60 years. 10 Our patient fi rst noticed skin papules when she was 57 years old, which represents a relatively late onset of the disease.…”