Malignant Atrophic Papulosis

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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
Lethal cutaneous and gastrointestinal
arteriolar thrombosis
Fatal cutaneointestinal syndrome
Thromboangiitis cutaneointestinalis
disseminata
Dermatite 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
Introduction
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Very rare disease (130-150
publications with approx. 200 cases)
●
No age or racial prevalence
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The skin lesions are pathognomonic
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The etiology is unclear
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No known effective therapy
Classification
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Vasculopathy / endovasculitis
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Occlusive arteriopathy
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Progressive, small- and mediumsize arterial occluding disease
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Leads to tissue infarction
●
It initially involves the skin (systemic
lesions may rarely precede skin lesions)
Systemic involvement
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 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
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
Etiology / Pathophysiology
●
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 (?)
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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%) !
Pinault AL et al (2004) Ann Dermatol Venereol 131:989-93
Differential diagnosis
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Lichen myxedematosus
●
Allergic vasculitis
●
Systemic Lupus erythematosus with
scar formation
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Papulonecrotic tuberculoid
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
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
New name proposal
Atrophic Papulosis (Köhlmeier-)Degos
Pathergy test
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Adamantiades-Behçet’s disease
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Pyoderma gaengrenosum
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Crohn’s disease
●
Rheumatoid arthritis
●
MAP ?
Etiology
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Vascular endothelium (?)
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Virus (serum / cells) (?)
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c-reactive protein (?)
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