An atypical paediatric case of malignant atrophic papulosis (Köhlmeier-Degos disease).
A new case of malignant atrophic papulosis (Köhlmeier-Degos disease) is reported. Vascular symptoms began at 17 months of age with cerebral ischaemia and progressive involvement of fingers and toes with torpid ulcers and apical necrotic amputations. At 6 years of age he developed chronic intestinal ischaemia with malabsorption and a new cerebral attack; in spite of anti-aggregant therapy the disease progressed and he died 7 months after diagnosis from a third cerebral ictus. Since the typical skin lesions of the disease were absent, the diagnosis was made on the basis of a pathological pattern of an occluded biopsied artery. The elder brother presents clinical and instrumental vascular involvement without cutaneous lesions and could be slightly affected.
Barabino
A
A
III Paediatric Department G, Gaslini Scientific Institute, Genova, Italy.
Pesce
F
F
Gatti
R
R
Colotto
P
P
Nobili
F
F
Colacino
R
R
Giampalmo
A
A
D001157:Arterial Occlusive Diseases / Q000503:physiopathology*; D002545:Brain Ischemia / Q000503:physiopathology*; D002648:Child; D005385:Fingers / Q000473:pathology; D006801:Humans; D008286:Malabsorption Syndromes / Q000503:physiopathology*; D008297:Male; D011928:Raynaud Disease / Q000150:complications / Q000503:physiopathology; D012883:Skin Ulcer / Q000209:etiology