A fatal case of malignant atrophic papulosis (Degos' disease) in a man with factor V Leinden mutation and lupus anticoagulant.

Abstract

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.

• Authors: Thomas Hohwy, Martin Glümer Jensen, Anders Tøttrup, Torben Steiniche, Karsten Fogh
• Journal: Acta dermato-venereologica (Acta Derm Venereol) Vol. 86 Issue 3 Pg. 245-7 ( 2006) ISSN: 0001-5555 [Print] Sweden
• PMID: 16710585 (Publication Type: Case Reports, Journal Article)

Chemical References

• Lupus Coagulation Inhibitor
• factor V Leiden
• Factor V

Topics

• Adult
• Diagnosis, Differential
• Factor V (genetics)
• Fatal Outcome
• Humans
• Intestinal Perforation (complications, surgery)
• Lupus Coagulation Inhibitor (blood)
• Lupus Erythematosus, Systemic (blood, complications, diagnosis, pathology)
• Male
• Mutation
• Sepsis (complications)
• Skin Diseases, Papulosquamous (complications, diagnosis, pathology)