Benign Degos' disease developing during pregnancy and followed for 10 years.

[malignant atrophic papulosis]

Degos ' disease , or malignant atrophic papulosis , is a rare and often fatal multisystem vasculopathy of unknown etiology . The cutaneous manifestations comprise erythematous papules , which heal to leave scars with a pathognomonic central porcelain-white atrophic area and a peripheral telangiectatic rim . Involvement of the gastrointestinal tract is observed in 50 % of cases , with intestinal perforation being the most common cause of death . Other organ systems can also be affected ; 20 % of cases involve the central nervous system . Systemic manifestations usually develop from weeks to years after onset of skin lesions or , in rare instances , may precede skin lesions . In the patient with Degos ' disease reported in this article , the characteristic skin lesions developed during pregnancy , a precipitating event not previously reported . She has survived an unusually long time (10 years ) without visceral or neurological involvement , despite florid cutaneous lesions . Moreover , we could detect the presence of antiphospholipid antibodies , the significance of which are currently unclear . These observations therefore confirm that there may be a strictly cutaneous form of Degos ' disease with a favourable prognosis .

Diseases
Validation

Diseases presenting "common cause" symptom

achondroplasia

acute rheumatic fever

adrenomyeloneuropathy

allergic bronchopulmonary aspergillosis

alpha-thalassemia

aniridia

aromatase deficiency

benign recurrent intrahepatic cholestasis

cadasil

child syndrome

coats disease

congenital adrenal hyperplasia

congenital toxoplasmosis

cushing syndrome

erdheim-chester disease

esophageal adenocarcinoma

esophageal squamous cell carcinoma

fabry disease

familial hypocalciuric hypercalcemia

hydrocephalus with stenosis of the aqueduct of sylvius

inclusion body myositis

junctional epidermolysis bullosa

kabuki syndrome

lamellar ichthyosis

legionellosis

liposarcoma

locked-in syndrome

malignant atrophic papulosis

neonatal adrenoleukodystrophy

neuralgic amyotrophy

pendred syndrome

primary hyperoxaluria type 1

pyruvate dehydrogenase deficiency

scrub typhus

systemic capillary leak syndrome

thoracic outlet syndrome

typhoid

von hippel-lindau disease

wiskott-aldrich syndrome

zellweger syndrome

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