Cases reported "Urticaria Pigmentosa"

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1/47. Cutaneous and systemic manifestations of mastocytosis.

    mastocytosis is characterized by an excessive number of apparently normal mast cells in the skin and, occasionally, in other organs. Characteristic skin lesions, called urticaria pigmentosa, are present in most patients, but clinical presentation can vary from a pruritic rash to unexplained collapse and sudden death. These lesions are typically tan to red-brown macules that appear on the trunk and spread symmetrically. patients with mastocytosis often have a long history of chronic and acute symptoms that were unrecognized as mastocytosis. skin lesions may or may not accompany systemic mastocytosis. Systemic disease may involve the gastrointestinal tract, the bone marrow or other organs. Even when the disease is considered as a possibility by the physician, the diagnosis can be difficult because of special technical requirements necessary for biopsy and because of the problems with biochemical testing. drug therapy is initiated to stabilize mast cell membranes, to reduce the severity of the attacks and to block the action of inflammatory mediators. The mainstay of therapy is histamine H1 and H2 blockers and the avoidance of triggering factors.
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2/47. Electron microscopic findings in a case of systemic mastocytosis.

    For several decades urticaria pigmentosa had been considered to be a benign and purely cutaneous disease, caused by infiltrations of tissue mast cells in the skin. Yet until 1962 at least 24 cases of systemic mastocytosis had been published. Sagher estimated the incidence of systemic mastocytosis as being in the region of about 10% of all cases of urticaria pigmentosa. A case of systemic mastocytosis in a 4-year-old child is described. biopsy specimens were examined by electron microscopy to study the process of degranulation of tissue mast cells during which histamine and heparin are released.
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3/47. Combined melanocytoma-mastocytoma in a case of nodular mastocytosis.

    A patient with long-standing nodular mastocytosis developed a slate-blue lesion on the scalp which produced symptoms of histamine release on mechanical and thermal stimulation. light and electron microscopic examination of the lesion showed zones of mast cells and zones of melanized cells. A transition zone showing cells with dual (melanosomal and mast) granulation, as well as granules representing intergrades between classical mast granules and melanosomes, was present. Features of this lesion add to the accumulated evidence that there is a histogenetic relationship between mast cells and melanocytes.
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4/47. tetany, malabsorption, and mastocytosis.

    mastocytosis is characterized by increased proliferation of mast cells. Two patients had systemic mastocytosis involving the skin and gastrointestinal tract, complicated by malabsorption and tetany. absorption studies in these patients suggested that the entire small bowel was involved and that the defect was mild in the absence of diarrhea. Small bowel biopsies disclosed infiltration of the lamina propria and submucosa by mast cells, and gastrointestinal tract x-ray films showed nodular densities, edema, and thickening of the bowel wall. tetany was due in part to combined hypocalcemia, hypomagnesemia, and hypokalemia. diarrhea and malabsorption were due to mast cell infiltration of the bowel rather than to histamine. patients with signs of systemic mastocytosis should have careful evaluations and be followed up to prevent development of malabsorption and tetany.
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5/47. Severe human urticaria produced by ant (Odontomachus bauri, Emery 1892) (hymenoptera: Formicidae) venom.

    BACKGROUND: Ant sting reactions are becoming an increasing problem in tropical countries. It is important for physicians to be aware of their possible cutaneous and systemic manifestations. methods: A child with a severe allergic reaction to an ant sting was examined and is discussed. This is the first case described in the literature caused by the ant Odontomachus bauri, and the biology of the ant and the clinical signs in the patient are described. RESULTS: A child developed a strong allergic reaction to an ant sting. The case was not associated with a previous ant sting. Treatment with the systemic antihistamine loratadine (Clarityn) was favorable in this patient. CONCLUSIONS: In tropical areas, ant stings are usually benign, self-limited pathologic processes. In some cases, however, severe allergic reactions can develop, including urticaria and anaphylactic shock. physicians should be aware of the possible complications of ant stings.
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6/47. Mithramycin in the treatment of systemic mastocytosis.

    A patient had systemic mastocytosis of 16 years' duration, with urticaria pigmentosa, osteopetrosis with severe bone pain, mastocytosis of the bone marrow with myeloid metaplasia, gastrointestinal malabsorption, and Cushingold features associated with prolonged usage of corticosteroids. Treatment with a single dose of mithramycin resulted in an abrupt disappearance of the patient's generalized bone pain, but it had little effect on his histamine release-type symptomatology.
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7/47. Acute spinal epidural hematoma and systemic mastocytosis.

    BACKGROUND: Systemic mastocytosis is a mast cell proliferative disorder affecting many organs that is rarely associated with internal bleeding. OBJECTIVE: To describe a case of spinal epidural hematoma in a patient with past medical history of urticaria pigmentosa and osteoporosis diagnosed with systemic mastocytosis. CASE REPORT: A 63-year-old woman with urticaria pigmentosa was admitted to hospital for severe back pain after minor trauma. physical examination showed pain on pressing T12 and L1 spinous processes, bilateral Lasegue sign, absent ankle jerk, and extensor plantar response. Computed tomography disclosed L3 fracture, and magnetic resonance imaging revealed spinal epidural hematoma and T2 hyperintensive scattered vertebral foci that suggested malignancy. The 24-hour urine histamine was very high. Mast cell infiltration was found in bone marrow biopsy. Because power was normal and there was no clinical sphincter disorder, the patient was successfully treated with conservative care. CONCLUSIONS: To our knowledge, acute intraspinal epidural hematoma has never been associated with mastocytosis. The hematoma was likely related to the vertebral fracture as well as a hemorrhagic diathesis due to anticoagulants released by local mast cells.
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8/47. Erythrodermic mastocytosis.

    Erythrodermic mastocytosis is a rare variant of diffuse cutaneous mastocytosis in which the skin becomes red, thickened, and lichenified and has a doughy consistency with multiple small papules on its surface, giving a leathery appearance to the skin. In this report, I describe a curious case of erythrodermic mastocytosis that appears to be due to vasodilation rather than to mast cell infiltration of the skin. In my opinion, this case might be an example of generalized telangiectasia macularis eruptiva perstans. Results of all laboratory tests failed to demonstrate systemic mast cell involvement; therapy with a combination of H1 and H2 antihistamines plus disodium cromoglycate controlled the symptoms.
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9/47. Neonatal mastocytosis with pachydermic bullous skin without c-Kit 816 mutation.

    BACKGROUND: Bullous mastocytosis is an unusual variant of mast cell disease with widespread bullae as the main cutaneous feature induced by mast cell proteases that cause dermoepidermal separation. CASE REPORT: A rare case of diffuse cutaneous bullous mastocytosis with pachydermia and unusually extensive skin folding is described in a 3-week-old girl. The diagnosis was confirmed by immunohistochemistry with Giemsa stain, the naphthol ASD chloroacetate esterase reaction and elevated blood levels for tryptase, histamine in serum and histamine and 1.4-methylimidazol acetic acid in the 24-hour urine. blood cell count was normal, as were thrombocytosis and leukocytosis. FACS analysis of the bone marrow aspiration material showed 1% mast cells. No c-Kit 816 [Asp-->Val] somatic mutation was found. Systemic involvement of other organs was excluded. DISCUSSION: The prognosis of c-Kit-negative diffuse bullous mastocytosis is not known. Regular blood controls are mandatory, and screening for germ cell ovarian cancer and bone marrow controls should be performed as well.
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keywords = histamine
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10/47. Anesthetic management of a labouring parturient with urticaria pigmentosa.

    PURPOSE: To report the anesthetic management of labour pain and cesarean section in a patient with urticaria pigmentosa at risk for systemic mastocytosis. CLINICAL: A 37-yr-old patient with a history of urticaria pigmentosa and an allergic reaction to a local anesthetic agent was seen in consultation at 36 weeks gestation. She previously tested negative for an allergy test to lidocaine. Recommendations to avoid systemic mastocytosis included: avoidance of histamine-releasing drugs, using lidocaine for labour epidural, and regional anesthesia in case of a cesarean section. The patient presented at term in labour. Intravenous fentanyl was used for early labour, followed by a combined spinal-epidural. The spinal contained lidocaine and fentanyl, but because of pruritus, the epidural infusion contained lidocaine only. Most likely because of tachyphylaxis to lidocaine, an epidural bolus of lidocaine with epinephrine failed to provide adequate anesthesia for a cesarean section. The block was supplemented with nitrous oxide by mask, with fentanyl postdelivery. Postoperative pain control was managed with an epidural infusion of lidocaine and fentanyl for three days. The patient was discharged without complications four days postsurgery. CONCLUSION: Proper allergy testing prior to pregnancy is important to help the management of labour pain and anesthesia for cesarean section in a patient at risk for systemic mastocytosis.
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