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urticaria pigmentosa/diare
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Urticaria pigmentosa: a clinical, hematopathologic, and serologic study of 30 adults.
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Urticaria pigmentosa (UP) is the most common form of cutaneous mastocytosis and may be associated with systemic involvement, most often of the bone marrow. The incidence of systemic involvement is not yet well established, however. To address this question, we subjected a group of 30 adults with
Severity of cutaneous findings predict the presence of systemic symptoms in pediatric maculopapular cutaneous mastocytosis.
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Although the prognosis of maculopapular cutaneous mastocytosis (MPCM), also referred to as urticaria pigmentosa, is often benign, clinicians lack evidence to reliably predict those at risk of associated systemic manifestations. We sought to elucidate clinical markers of disease severity to provide
A 1-year-old boy with persistent, generalized eruption. Urticaria pigmentosa.
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A healthy 1-year-old boy born at full term after an uncomplicated pregnancy presented to the dermatology clinic for a widespread eruption with gradual onset since 3 months of age. He was otherwise well, afebrile, feeding well, and gaining weight appropriately. The child was overall asymptomatic,
Type Ib indolent mastocytosis with systemic involvement: cutaneous mastocytosis and gastrointestinal involvement at young girl.
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A 21-year-old young girl presents with intense abdominal pain, nausea, diarrhea in the context of a cutaneous eruption formed by erythematous and papulous elements with brown violet aspect, very pruriginous, occasioned by the preparation of some fishmeal. Similar eruption debuted from childhood from
c-kit mutation and osteopetrosis-like osteopathy in a patient with systemic mast cell disease.
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We describe the case of a 69-year-old man with systemic mastocytosis and severe osteopetrosis who carries a somatic activating mutation in the c-kit proto-oncogene. The patient initially presented with urticaria pigmentosa, progressing to systemic mast cell disease with severe anemia due to bone
Gastrointestinal manifestations of systemic mastocytosis.
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Systemic mastocytosis (SM) is a rare disease with abnormal proliferation and infiltration of mast cells in the skin, bone marrow, and viscera including the mucosal surfaces of the digestive tract. Gastrointestinal (GI) symptoms occur in 14%-85% of patients with systemic mastocytosis. The GI symptoms
Hypotension, Syncope, and Fever in Systemic Mastocytosis without Skin Infiltration and Rapid Response to Corticosteroid and Cyclosporin: A Case Report.
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Mast cell disorders are defined by an abnormal accumulation of tissue mast cells in one or more organ systems. In systemic mastocytosis, at least one extracutaneous organ is involved by definition. Although, systemic mastocytosis usually represents with skin lesion called urticaria pigmentosa, in a
Mastocytosis: one year's experience.
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The diagnosis of systemic mastocytosis without urticaria pigmentosa has been made with increasing frequency since modern methods of histamine assay have been used clinically. We examined the incidence of urticaria-angioedema and mastocytosis over a recent 12-month period. Of 490 new patients we saw,
Mastocytosis presenting as a skeletal disorder.
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Mastocytosis is a rare disease of mast-cell proliferation with involvement of the reticuloendothelial systems including skin, bone, gastrointestinal tract, liver, lungs, spleen, and lymph nodes. Systemic mastocytosis is characterized by a combination of symptoms that relate to the mast cells'
Mastocytosis in children: clinicopathological study based on 35 cases.
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Immunohistochemical staining is useful in the diagnosis of bone marrow infiltration in systemic mastocytosis. However, it is not clear if antibody staining may be helpful in the diagnosis of cutaneous mastocytosis (CM). We studied the histological appearance of CM in 35 pediatric patients. Cases
Current approaches to the diagnosis and treatment of systemic mastocytosis.
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OBJECTIVE
To review the clinical manifestations of mastocytosis and examine the recommended diagnostic procedures and therapeutic options available for the treatment of this condition.
METHODS
PubMed searches were performed for articles published regarding presentation and classification of
Gastroenterologic manifestations of mastocytosis.
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Mastocytosis as a rare disease is usually diagnosed by dermatologists because of urticaria pigmentosa as one of the leading signs. If the triad of urticaria pigmentosa, flush and diarrhea is present diagnosis is easily made. However, urticaria pigmentosa and flush may be less dominant or even absent
An unproven technique with potentially fatal outcome: provocation/neutralization in a patient with systemic mastocytosis.
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OBJECTIVE
To describe the risks associated with use of an unproven technique, provocation/neutralization, in diagnosis and treatment of a putative "food allergy" in a patient with systemic mastocytosis.
METHODS
A case report of a 68-year-old woman with mastocytosis is reported. The patient was
[Clinical variability in two cases of systemic mastocytosis].
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Two cases of systemic mastocytosis with different clinical course were described. The first of them manifested with returning attacks of tachycardia with raise of blood pressure, followed by its drop and loss of consciousness. Abdominal pain and persistent diarrhea characterized the second one. The
[Systemic mast cell disease associated with cutaneous xanthomas and markedly elevated serum IgE].
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Systemic mast cell disease (SMCD) is a rare disease often associated with symptoms of general malaise, pruritus, diarrhea, vomiting, fever, urticaria pigmentosa, hepatosplenomegaly and lymphadenopathy. We reported a case of SMCD associated with cutaneous xanthoma and serum hyper IgE. Skin biopsy
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