1 / 20

Malignant Atrophic Papulosis A clinical synopsis Christos C. Zouboulis

Malignant Atrophic Papulosis A clinical synopsis Christos C. Zouboulis. Degos Disease (DD) Malignant atrophic papulosis (MAP) Papulosis atrophicans maligna Köhlmeier-Degos-Delort-Tricot syndrome Köhlmeier-Degos syndrome Köhlmeier-Degos' disease Papuleuse maligne atrophiante

jacqui
Télécharger la présentation

Malignant Atrophic Papulosis A clinical synopsis Christos C. Zouboulis

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. Malignant Atrophic PapulosisA clinical synopsis Christos C. Zouboulis

  2. Degos Disease (DD) Malignant atrophic papulosis (MAP) Papulosis atrophicans maligna Köhlmeier-Degos-Delort-Tricot syndrome Köhlmeier-Degos syndrome Köhlmeier-Degos' disease Papuleuse maligne atrophiante Lethal cutaneous and gastrointestinal arteriolar thrombosis Fatal cutaneointestinal syndrome Thromboangiitis cutaneointestinalis disseminata Dermatite papulosquameuse atrophiante Online Mendelian Inheritance in Man (OMIM): 602248

  3. Köhlmeier W (1941) Multiple Hautnekrosen bei Thrombangiitis obliterans. Arch Dermatol Syphilol (Wien) 181:783-92 Degos R, Delort J, Tricot R (1942) Dermatite papulosquameuse atrophiante. Bull Soc Fr Derm Syph 49:148-50 Degos R, Delort J, Tricot R (1948) Papulose atrophiante maligne (syndrome cutanéo-intestinal mortel). Bull Mém Soc Med Hôp Paris 64:803-6 Lausecker H (1949) Beitrag zur intestinalen Form der Thrombangiitis obliterans mit Hauterscheinungen. Acta Derm Venereol (Stockh) 29:369-87

  4. Introduction • Very rare disease (130-150 publications with approx. 200 cases) • No age or racial prevalence • The skin lesions are pathognomonic • The etiology is unclear • No known effective therapy

  5. Classification • Vasculopathy / endovasculitis • Occlusive arteriopathy • Progressive, small- and medium-size arterial occluding disease • Leads to tissue infarction • It initially involves the skin (systemic lesions may rarely precede skin lesions)

  6. Systemic involvement Gastrointestinal tract:abdominal pain, nausea, vomiting, diarrhoea or constipation and, in the later stages, intestinal perforation and haemorrhage

  7. Systemic involvement Neurological involvement:peripheral and central nervous system headache, dizziness, seizures, hemiplegia (total or partial paralysis of one side of the body), aphasia (loss or impairment of the power to use or comprehend words), paraplegia (paralysis of the lower half of the body), gaze palsy (partial or complete inability to move the eyes to all directions of gaze)

  8. Systemic involvement Other organs Cardiac: Pleuritis, Pericarditis Eye: eyelid ptosis (drooping of the upper eyelid), optic neuritis (inflammation of optic nerves), diplopia (double vision), visual field defectsLungs, kidneys, bladder, liver, pancreas

  9. High WA et al (2004) Is Degos' disease a clinical and histological end point rather than a specific disease? J Am Acad Dermatol 50:895-9 Ball E et al (2003) Degos' disease: a distinctive pattern of disease, chiefly of lupus erythematosus, and not a specific disease per se. Am J Dermatopathol 25:308-20

  10. Etiology / Pathophysiology • Unknown • No circulating immune complexes, antiendothelial cell antibodies, anticardiolipin antibodies (in sime cases antiphospholipid antibodies detected) • No medication and toxic chemicals

  11. Etiology / Pathophysiology • Disturbance of immunity • Viral infection (no paromyxovirus) • Abnormality of the blood clothing system • Primary endothelial cell defect

  12. Etiology / Pathophysiology • Primary endothelial cell defect with secondary thrombosis (?) • Partially impaired fibrinolytic activity and alterations in platelet function • No inflammation !

  13. Etiology / Pathophysiology • Familial cases: Autosomal dominant mode of inheritance (?) • Are familial cases more benign than sporadic ones ?32 cases in 10 families: 4 cases with malignant course (12.5%) ! Pinault AL et al (2004) Ann Dermatol Venereol 131:989-93

  14. Differential diagnosis • Lichen myxedematosus • Allergic vasculitis • Systemic Lupus erythematosus with scar formation • Papulonecrotic tuberculoid

  15. Course and prognosis • Malignant (systemic involvement) • Benign (cutaneous involvement only) Mensing C, Mensing H (2002) Degos atrophic malignant papulosis. Not always malignant! Hautarzt 53:42-6

  16. Course and prognosis • No controlled study • Benign form: Thrombocyte aggregation inhibitors- Pentoxifylline 1200 mg/d- Acetyl salicylic acid 100 mg/d and/or dipyridamole- Corticosteroids

  17. New name proposal Atrophic Papulosis (Köhlmeier-)Degos

  18. Pathergy test • Adamantiades-Behçet’s disease • Pyoderma gaengrenosum • Crohn’s disease • Rheumatoid arthritis • MAP ?

  19. Etiology • Vascular endothelium (?) • Virus (serum / cells) (?) • c-reactive protein (?)

More Related