[Diffuse plane xanthoma with arthritis, serositis, erythema nodosum, vasculitis and myelomonocytic leukemia. Description of a case with autopsy].
A 57-year-old woman consulted for a severe xanthelasma followed by a severe and complex systemic disease and died ten years later with xanthomas extended to the face and trunk. Her lipid and cholesterol levels were normal but HDL was below normal values. Diabetes as well as amyloidosis, paraproteinemia and Bence Jones protein in urine were not found. Three years after her initial consultation monocytosis appeared in most but not all of her hemograms. Soon after her spleen was palpable and became very large. She had three bone marrow biopsies showing progressive white cell hypercellularity that was considered as possibly reactive. During her long illness she had recurrent conjunctivitis and systemic complications such as arthritis, pleural and pericardial effusions, vasculitis, sudden deafness with Ménière-like vertigo, erythema nodosum and myositis that responded, at least partially, to steroid treatments though she remained corticoid dependent in order to control her more severe symptoms. Those complications were considered to be probably due to vasculitis or immune phenomena. After a long follow up it was clear that her disease was better classified as diffuse plane xanthoma (DPX) related to a myeloproliferative syndrome and vasculitis. Her final hospitalization was due to a severe esophageal moniliasis and she died a few days later. An autopsy was performed and a chronic myelomonocytic leukemia was the final diagnosis. The ultimate cause of death was a perforated acute duodenal ulcer with peritonitis. Possible relations between DPX, immunologic reactions and myelomonocytic leukemia are discussed.
Maxit
M J
MJ
Hospital Privado de Comunidad Córdoba 4545, 7600 Mar del Plata. ddi@hpc.org.ar
Paz
R A
RA
D001168:Arthritis / Q000150:complications; D004893:Erythema Nodosum / Q000150:complications; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D015477:Leukemia, Myelomonocytic, Chronic / Q000150:complications / Q000473:pathology*; D008875:Middle Aged; D012700:Serositis / Q000150:complications; D014657:Vasculitis / Q000150:complications; D014973:Xanthomatosis / Q000150:complications / Q000473:pathology*