[Familial Degos' atrophic papulosis (mother-daughter)].
Degos' disease (malignant atrophic papulosis) occurring in mother and daughter is described. Mother's eruption is composed of multiple characteristic lesions (up to 600). She does not have any gastrointestinal, neurological nor other visceral manifestations after a 9-year course. One year after the onset of the disease, she had a daughter. The pregnancy was normal and the newborn did not have any cutaneous manifestation. The daughter's first skin lesion appeared when she was 3-months old. She has now been under observation for nearly nine years and developed only four skin lesions without any visceral manifestation. Laboratory investigations show an increase in fibrinogen in both mother and child, without any sign of disseminated intravascular coagulation. The research for "slow virus" by a primate's intravascular inoculation of mother's skin lesion in negative.
D000328:Adult; D001284:Atrophy / Q000235:genetics; D005260:Female; D005340:Fibrinogen / Q000032:analysis; D006801:Humans; D007223:Infant; D012867:Skin / Q000473:pathology*; D012871:Skin Diseases / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology
D005340:Fibrinogen
Papulose atrophiante de Degos familiale (mére-fille).