A fatal case of malignant atrophic papulosis (Degos' disease) in a man with factor V Leinden mutation and lupus anticoagulant.
Malignant atrophic papulosis (Degos' disease) is a very rare condition characterized by atrophic papular skin lesions and variable association of systemic involvement. We describe a 33-year-old man who presented with a widespread skin eruption consistent with malignant atrophic papulosis. During the course of the disease he even developed penile ulcerations, a symptom that has been reported only a few times previously. He subsequently died of multiple perforations of the small bowel 2.5 years after onset of the disease. Laboratory investigations revealed a mutation of factor V Leiden and the presence of lupus anticoagulant, but no anti-cardiolipin antibodies. The patient was treated with narrow-band ultraviolet (UV)B, prednisolone and, later, aspirin, pentoxifyllin and warfarin. Despite this very intensive anticoagulant and anti-platelet therapy, the treatment had no effect on the skin lesions and could not prevent systemic involvement.
Hohwy
Thomas
T
Department of Dermatology, Aarhus University Hospital, Denmark. thomashohwy@hotmail.com
Jensen
Martin Glümer
MG
Tøttrup
Anders
A
Steiniche
Torben
T
Fogh
Karsten
K
D000328:Adult; D003937:Diagnosis, Differential; D005165:Factor V / Q000235:genetics; D017809:Fatal Outcome; D006801:Humans; D007416:Intestinal Perforation / Q000150:complications / Q000601:surgery; D016682:Lupus Coagulation Inhibitor / Q000097:blood; D008180:Lupus Erythematosus, Systemic / Q000097:blood / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D008297:Male; D009154:Mutation; D018805:Sepsis / Q000150:complications; D017444:Skin Diseases, Papulosquamous / Q000150:complications / Q000175:diagnosis* / Q000473:pathology
D016682:Lupus Coagulation Inhibitor; C095381:factor V Leiden; D005165:Factor V