[Systematized papulosis of the lower scapular areas with ultrastructural anhist bodies: specific cutaneous symptoms of Hunter's disease (author's transl)].
Papulosis of the scapular areas is a characteristic dermatologic sign of Hunter's disease. It has not been described in the other mucopolysaccharidoses. This sign had been detailed by Hunter in 1917 in his princeps observation. We noted it in two cases of the disease. The ultrastructural study showed an accumulation in the connective interstitial substance of anhist bodies of intra-cellular origin.
D002648:Child; D003238:Connective Tissue / Q000473:pathology*; D006801:Humans; D008297:Male; D016532:Mucopolysaccharidosis II / Q000235:genetics / Q000473:pathology*; D010375:Pedigree
Papulose sous-scapulaire systématisée avec corps anhistes en microscopie électronique: signes cutanés spécifiques de la maladie de hunter.