Systemic mastocytosis with diffuse cutaneous involvement and haematological disease presenting in utero treated unsuccessfully with vincristine.

J Angus, I H Leach, J Grant, J C Ravenscroft

Journal: Clinical and experimental dermatology 2008;33(1):36-9

PMID: 17983456

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Abstract

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.

Address: Department of Dermatology, Queens Medical Centre, Nottingham, UK. [email protected]

DOI: 10.1111/j.1365-2230.2007.02555.x

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MeSH Terms: Antineoplastic Agents, Phytogenic, Ascites, Fatal Outcome, Female, Fetal Distress, Hepatomegaly, Humans, Infant, Newborn, Male, Mast Cells, Mastocytosis, Systemic, Pregnancy, Prenatal Care, Skin Diseases, Splenomegaly, Treatment Failure, Vincristine