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Malignant Atrophic Papulosis A clinical synopsis Christos C. Zouboulis.

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Presentation on theme: "Malignant Atrophic Papulosis A clinical synopsis Christos C. Zouboulis."— Presentation transcript:

1 Malignant Atrophic Papulosis A 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 ● 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 Introduction

5 ● 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) Classification


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

8 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) Systemic involvement

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

10 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

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

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

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

14 ● 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%) ! Etiology / Pathophysiology Pinault AL et al (2004) Ann Dermatol Venereol 131:989-93

15 ● Lichen myxedematosus ● Allergic vasculitis ● Systemic Lupus erythematosus with scar formation ● Papulonecrotic tuberculoid Differential diagnosis

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

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

18 Atrophic Papulosis (Köhlmeier-)Degos New name proposal

19 ● Adamantiades-Behçet’s disease ● Pyoderma gaengrenosum ● Crohn’s disease ● Rheumatoid arthritis ● MAP ? Pathergy test

20 ● Vascular endothelium (?) ● Virus (serum / cells) (?) ● c-reactive protein (?) Etiology

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