Malignant Atrophic PapulosisMalignant Atrophic PapulosisA clinical synopsisA clinical synopsis

Christos C. ZouboulisChristos C. Zouboulis

Degos Disease (DD)Malignant atrophic papulosis (MAP)Papulosis atrophicans malignaKöhlmeier-Degos-Delort-Tricot syndromeKöhlmeier-Degos syndromeKöhlmeier-Degos' diseasePapuleuse maligne atrophianteLethal cutaneous and gastrointestinal arteriolar thrombosisFatal cutaneointestinal syndromeThromboangiitis cutaneointestinalis disseminataDermatite papulosquameuse atrophiante

Online Mendelian Inheritance in Man (OMIM): 602248

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

● 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

● 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

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

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)

Systemic involvement

Other organsCardiac: 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

Systemic involvement

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

● Unknown

● No circulating immune complexes, antiendothelial cell antibodies, anticardiolipin antibodies (in sime cases antiphospholipid antibodies detected)

● No medication and toxic chemicals

Etiology / Pathophysiology

● Disturbance of immunity

● Viral infection (no paromyxovirus)

● Abnormality of the blood clothing system

● Primary endothelial cell defect

Etiology / Pathophysiology

● Primary endothelial cell defect with secondary thrombosis (?)

● Partially impaired fibrinolytic activity and alterations in platelet function

● No inflammation !

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%) !

Etiology / Pathophysiology

Pinault AL et al (2004) Ann Dermatol Venereol 131:989-93

● Lichen myxedematosus

● Allergic vasculitis

● Systemic Lupus erythematosus with scar formation

● Papulonecrotic tuberculoid

Differential diagnosis

● 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

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

Atrophic Papulosis (Köhlmeier-)Degos

New name proposal

● Adamantiades-Behçet’s disease

● Pyoderma gaengrenosum

● Crohn’s disease

● Rheumatoid arthritis

● MAP ?

Pathergy test

● Vascular endothelium (?)

● Virus (serum / cells) (?)

● c-reactive protein (?)

Etiology