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Degos-Touraine syndrome; malignant atrophic papulosis
Etiology:
- genetic disease of complement
Epidemiology:
- rare, 200 cases reported worldwide
- all ages affected
Pathology:
- arteritis involving small-medium size arteries
- tissue ischemia & infarction
- initially involves skin
- occurs both in a limited benign, cutaneous form & in a potentially lethal multiorgan, systemic variant [1]
Clinical manifestations:
- skin lesions generally first manifestation
- initially, erythematous, pink or red papules
- papules heal leaving scars with pathognomonic, central, porcelain white atrophic centers
- does not involve the face
- systemic manifestations usually develop weeks to years after onset of skin lesions
- GI tract affected in 50%
- intestinal perforation
- central nervous system involved in 20%
- systemic Degos disease is universally fatal [1]
Complications:
- intestinal perforation most common cause of death
- other causes of death include:
- intestinal infarction
- pleuropericardial pathology
- stroke
- hemorrhage Manangement:
- eculizumab
General
genetic syndrome (multisystem disorder)
References
- Scheinfeld NS, Elston DM
Medscape: Degos Disease
https://emedicine.medscape.com/article/1087180-overview