Systemic mastocytosis with diffuse cutaneous involvement and haematological disease presenting in utero treated unsuccessfully with vincristine.
Mastocytosis is a disorder characterised by abnormal mast cell proliferation. The diverse spectrum of clinical presentations is dependent on the tissues and organs involved. We report a rare case of aggressive systemic mastocytosis presenting in utero with diffuse cutaneous involvement, and haematological abnormalities. There is little published evidence to guide treatment.
Angus
J
J
Department of Dermatology, Queens Medical Centre, Nottingham, UK. janeteangus@hotmail.com
Leach
I H
IH
Grant
J
J
Ravenscroft
J C
JC
D000972:Antineoplastic Agents, Phytogenic / Q000627:therapeutic use*; D001201:Ascites / Q000209:etiology; D017809:Fatal Outcome; D005260:Female; D005316:Fetal Distress / Q000209:etiology* / Q000473:pathology; D006529:Hepatomegaly / Q000209:etiology; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D008407:Mast Cells / Q000473:pathology*; D034721:Mastocytosis, Systemic / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D011247:Pregnancy; D011295:Prenatal Care; D012871:Skin Diseases / Q000209:etiology* / Q000473:pathology; D013163:Splenomegaly / Q000209:etiology; D017211:Treatment Failure; D014750:Vincristine / Q000627:therapeutic use*
D000972:Antineoplastic Agents, Phytogenic; D014750:Vincristine