Mastocytosis

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Running Head : MASTOCYTOSIS

Mastocytosis : Symptoms , Diagnosis and Treatment

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Mastocytosis is a disease characterized by proliferation and accumulation of apparently normal mast cells in the skin and occasionally , in other organs . It can occur in two forms - cutaneous and systemic . Cutaneous (skin ) mastocytosis constitutes 90 percent of cases of mastocytosis that are not associated with a hematologic dis (Gruchalla , 1995 as cited by Alto and Clarcq , 1999 . Types of cutaneous mastocytosis include solitary mastocytoma , diffuse erythrodermic mastocytosis , paucicellular mastocytosis and urticaria pigmentosa . Urticaria pigmentosa is

the most common type and is characterized by oval or round red-brown macules , papules , or plaques that range in number from a few to thousands (Hogan and Lewis , 2006 The macules initially appear at the trunk and rapidly spread symmetrically and centripetally (Alto and Clarcq , 1999 . The palms soles , face and scalp are the parts not usually affected . Exposure to certain environmental stimuli such as heat , cold or pressure usually results to localized urticaria (Darier 's sign . When the macules heal they usually do not produce any scarring unless superinfection occurs

While cutaneous mastocytosis involve only the skin , systemic mastocytosis is characterized by mast cell infiltration of extracutaneous organs (Krishnan and Ramu , 2006 . Before 1949 mastocytosis was thought to manifest only in the skin . Ellis was the first to establish an autopsy proving that the disease also involved other organs . An autopsy of a 1-year-old infant revealed that mastocytosis occurred in the bone marrow , lymph nodes , spleen , kidneys and pancreas . At present , mast cell infiltration has also been observed in the kidney and in the gastrointestinal (GI ) tract

The most obvious manifestation of mastocytosis is the appearance of macules , papules , nodules and plaques in the skin . Mast cells however produce chemicals (histamine being one of the most significant ) that change the body 's functioning and a large number of

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mast cells in the body result in abdominal pain , vomiting and diarrhea severe enough to cause malabsorption (Alto and Clarcq , 1999 . Headache hypotension leading to syncope , bleeding diathesis , peptic ulcer and even psychological changes such as irritability and difficulty in concentrating are also symptoms associated with the dis . In patients with systemic mast cell disease , GI symptoms are usually exhibited . Abdominal pain is the most common GI symptom , while diarrhea , nausea and vomiting follow abdominal pain in rank respectively (Krishnan and Ramu , 2006 . There are also symptoms and signs of gastroesophageal reflux disease noted in some cases . Symptoms of pruritus and flushing and signs of urticaria are also exhibited especially when the mast cell accumulation is associated with cutaneous abnormalities . If the skeletal system is involved , mastocytosis can be manifested as bone pain or the onset of a new bone fracture (Hogan and Lewis , 2006 . Involvement of the central nervous system may result in neuropsychiatric symptoms and in nonspecific changes such as malaise or irritability

Systemic mast cell disease has also been associated with a number of hematologic diseases , including hypereosinophilic syndrome , Castleman disease , monoclonal gammopathy , and hairy cell leukemia . Certain...